COLUMBIA  LIBRARIES  OFFSITE 

.HEAL.™  SCIENCES  STANDARD 


HX00052892 


■  •;  .  ■ 


: ..; 


RECAP 


:;     ■  ::.:: 


;.: 


■     .,.. 


^ase  Hospital  No.  20; 


RCiHl 


c  733. 


i/.  / 


Columbia  <Bmt>er*iti> 

COLLEGE  OF 

PHYSICIANS  AND  SURGEONS 

LIBRARY 


-«se  Hospital  No.  20 


Digitized  by  the  Internet  Archive 

in  2010  with  funding  from 

Open  Knowledge  Commons 


http://www.archive.org/details/textbookonnervou01asch 


TEXT-BOOK 

ON 

NERVOUS  DISEASES 
BURR 


miS 


Base  Hospital  No.  20, 

TEXT-BOOK 

ON 

NERVOUS  DISEASES 


BY 
G.  ASCHAFEENBURG,  COLOGNE;  H.  CURSCHMANN,  MAYENCE;  R.  FlNKELN- 

burg,  Bonn;  R.  Gaupp,  Tubingen;  C.  Hirsch,  Gottingen;  Fr.  Jamin, 
Erlangen;  J.  Ibrahim,  Munich;  Fedor  Krause,  Berlin;  M.  Lewan- 
dowsky,  Berlin;  Ff.  Liepmann,  Berlin;  L.  R.  Muller,  Augsburg; 
H.  Schlesinger,  Vienna;  S.  Schoenborn,  Heidelberg;  Ff.  Starck, 
Karlsruhe;  Ff.  Steinert,  Leipsic 


AUTHORIZED  ENGLISH  EDITION 
EDITED  by 

CHARLES  W.  BURR,  B.  S.,  M.  D. 

PROFESSOR   OF  MENTAL  DISEASES  IN  THE  UNIVERSITY  OF  PENNSYLVANIA; 
NEUROLOGIST  TO  THE  PHILADELPHIA  GENERAL   HOSPITAL 


WITH  156  TEXT  ILLUSTRATIONS 


Base  Hospital  No.  20; 
VOL.  I 


PHILADELPHIA 

P.   BLAKISTON'S   SON   &   CO. 

1012  WALNUT  STREET 


Copyright,  1915,  by  P.  Blakiston's  Son  &  Co. 

v.  | 


THE. MAPLE. PRESS. YORK. PA 


PREFACE  TO  THE  AMERICAN  EDITION 


The  German  contributors  to  this  book  should  need  no  introduction 
to  American  physicians  as  they  are  all  men  of  established  reputation.  The 
articles  are  based  on  personal  knowledge  and  experience  and  each  writer 
presents  his  own  well  thought  out  and  mature  opinions.  While  the  work 
is  purely  scientific  in  tone  and  conservative  in  attitude,  a  matter  of  much 
importance  in  these  days  when  much  wild  doctrine,  especially  as  to  mental 
therapeutics,  is  being  offered  the  public,  it  has  never  been  forgotten  by  the 
writers  that  the  only  useful  knowledge  is  that  which  leads  to  sane  therapeu- 
tics. There  is  no  need  to  discuss  the  advantages  offered  by  a  book 
written  by  many  authors  over  a  one-man  work  as  it  has  been  done  by  the 
editor  of  the  German  edition.  The  translators  have  endeavored  to  do 
their  work  faithfully  and  to  give  an  accurate  rendering  of  the  writers' 
statements.  The  American  editor  has  not  cut  out  the  many  references  to 
European  health  resorts,  not  because  he  thinks  Americans  need  go  abroad 
for  such  treatment,  but  in  order  to  encourage  Americans  to  develop  and 
encourage  their  own  natural  cure  places. 

Editor  of  the  American  Edition. 


PREFACE  TO  THE  GERMAN  EDITION 


The  marvellous  advance  made  by  neuro-therapeutics  during  the  second 
half  of  the  last  century  has,  as  a  natural  consequence,  induced  specialization 
within  the  confines  of  this  domain.  On  the  one  hand  this  tendency  was 
furthered  by  the  unlike  scientific  descent  of  the  leading  investigators,  among 
whom  from  the  very  first,  there  was  a  clear  cut  distinction  between  the 
psychiatrical-  and  the  interno-neurologists;  on  the  other  hand,  neurology, 
in  itself,  was  a  field  to  which  not  a  few  borderland  fields  naturally  attached 
themselves. 

Thus  it  has  come  to  pass  that  the  neuro-therapeutics  of  to-day  stands 
in  intimate  relation  not  only  with  psychiatry  and  internal  medicine,  but 
also  with  ophthalmology  and  otology,  with  dermatology,  bacterio-serology, 
and  above  all  with  surgery,  making  use  more  and  more  of  the  aid  afforded 
by  these  special  sciences. 

If  therefore  we  venture  to  submit  to  physicians  and  students  a  newly 
compiled  text-book  of  neuro-therapeutics,  we  are  not  blindly  following  the 
fashion — a  fashion  which  in  recent  years  has  given  extraordinary  precedence 
to  this  cooperative  method  of  text-book  production — but  are  conforming  in 
no  slight  degree  to  that  very  peculiarity  which  characterizes,  as  we  have 
said,  our  field.  Our  intention  was  to  produce  for  students  and  practitioners 
— that  is  essentially  for  non-specialists — a  text-book  comprising  old  material, 
that  has  long  been  established,  as  well  as  the  new,  nay  the  very  latest  ac- 
quisitions of  neuro-therapeutics.  It  aimed  to  give  those  who  have  but 
little  time  for  specialization  a  book  containing  what  is  essential,  concise, 
and  vitally  instructive,  though  naturally  devoid  of  that  fullness  to  be  found 
in  the  special  monographs.  How  could  these  requirements  be  better  met 
than  by  having  the  separate  chapters  written  by  authors  whose  work  and 
original  successful  researches  prove  that  they  stand  in  the  very  midst  of  the 
events  and  the  literature  of  each  field  concerned  ?  Within  the  narrow  con- 
fines of  a  text-book,  they  are  able  to  unite,  first  of  all,  brevity  with  clearness, 
and  completeness  with  the  necessary  conciseness.  A  few  examples  may 
serve  to  illustrate:  Clinical  treatment  of  disturbances  of  the  circulation  of 
the  brain,  however  much  it  concerns  therapeutically  the  special  neurologist, 
considering  its  close  relation  to  the  pathology  of  the  heart  and  the  circula- 
tion, doubtless  demands  presentation  by  an  internist  whose  special  subject 
is  the  pathologic  physiology  of  the  circulation.  The  discussion  of  menin- 
gitis, also,  will  be  less  adapted  to  the  special  neurologist  than  to  the  internist 
skilled  in  bacteriology  and  cyto-diagnosis.     On  the  other  hand,  the  exposi- 


Vlll 


PREFACE 


tion  of  the  present  condition  of  the  pathologic  physiology  of  the  brain  necessi- 
tates, in  view  of  its  complicated  structure,  and  the  rapid  progress  made  in 
recent  times,  a  specialist's  treatment.  It  is  no  accident  that  this  difficult 
realm  is  almost  or  quite  a  terra  incognita  to  many  interno-neurologists  of 
the  well  known  schools  and  that  it  is  to  a  small  degree  only  cultivated  by 
many  representatives  of  clinical  psychiatry.  And  finally:  of  all  the  neurol- 
ogists, internists  and  psychiatrists,  who  should  undertake  to  present  briefly 
and  critically  the  modern  status  as  to  the  operative  possibilities  in  neuro- 
therapeutics  ?  Every  one  will  here  give  precedence  to  the  surgeon  of  this 
special  field,  and  we  are  glad  and  proud  to  have  found  as  collaborator  for 
this  special  field  the  most  noted  German  brain  surgeon. 

I  think  that  I  have  shown  by  these  few  examples  that  the  compiling  of  a 
text-book  that  recognizes  the  newly-acquired  results,  by  several  authors, 
may  well  be  justified.  That  disadvantages  may,  to  some  extent,  offset  the 
advantages  of  this  method,  can  not  be  denied,  and  corresponds  to  the  experi- 
ence with  compiled  text-books  in  other  fields  of  medicine.  On  account  of 
the  multiplicity  of  authors,  the  uniformity  of  presentation  necessarily  suffers, 
nor  can  repetitions  be  altogether  avoided.  We  ask  the  reader,  therefore, 
to  judge  these  faults  leniently,  and  hope  to  obviate  them  to  some  extent  in 
later  editions. 

Should  we  succeed  in  arousing  by  this  text-book,  in  wider  classes  of  non- 
specializing  physicians,  interest  for  and  understanding  of  neurology  as  an 
equally  justifiable  and  important  special  field  among  those  other  special 
fields  long  officially  recognized  as  being  of  signal  importance,  we  shall  see 
in  this  success  at  once  the  attainment  of  our  purpose  and  our  reward. 

The  Editor. 


TABLE  OF  CONTENTS 


i 

GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

By  Privatdocent  Dr.  S.  Schoenborn,  Heidelberg 

Pagb 

i.  Taking  the  anamnesia i 

2.  The  objective  findings 3 

A.  General  remarks 3 

B.  Testing  the  sense  organs,  voice  and  speech 5 

1.  The  eye 5 

2.  The  ear 14 

3.  Taste  and  smell      16 

4.  Disturbances  in  voice  and  speech 17 

5.  Disturbances  in  the  realm  of  the  other  cerebral  nerves 20 

C.  Motility 21 

D.  Sensibility 54 

E.  Reflexes 64 

F.  Vasomotor,  trophic,  and  secretory  disturbances 72 

G.  Examination  of  the  cerebro-spinal  fluid 75 

H.  Examination  with  Rontgen  rays 77 

II 

DISEASES  OF  THE  PERIPHERAL  NERVES 
By  Privatdocent  Dr.  H.  Steinert,  Leipsic 

Introductory  anatomical  remarks  and  definition 78 

Classification  of  peripheral  nerve  diseases 80 

I.  Destructive  diseases  of  the  peripheral  nerves 80 

A.  General  part 80 

1.  General  pathological  anatomy 80 

2.  General  symptomatology  and  pathological  physiology     .....  83 

3.  General  diagnostics  and  prognostics 89 

4.  Division  of  the  destructive  diseases 93 

B.  Special  part 94 

Diseases  of  single  peripheral  nerves 94 

General  aetiology 94 

Pathological  anatomy 95 

General  therapeutics 98 

Paralyses  of  single  peripheral  nerves 106 

A.  The  cranial  nerves 106 

1.  The  olfactory  nerve .' 106 

2.  The  optic  nerve 107 

ix 


x  COX  TEXTS 

Page 

3.  Nerves  of  the  eye  muscles.     Oculomotor,  trochlear  and  abducens 

nerves •  II2 

4.  The  trigeminal  nerve 119 

5.  The  facial  nerve 123 

6.  The  auditory  nerve 133 

7.  The  glossopharyngeal  nerve 137 

8.  The  vagus  (pneumogastric)  nerve 137 

9.  The  accessory  nerve 14° 

10.  The  hypoglossal  nerve 142 

B.  The  spinal  nerves      143 

1.  The  phrenic  nerve 144 

2.  The  brachial  plexus  and  its  individual  nerves 145 

3.  The  other  thoracic  nerves 157 

4.  Xerves  of  the  lumbar  and  sacral  plexuses 157 

5.  Xerves  of  the  pudendal  and  coccygeal  plexuses 164 

Supplement:  Occupational  paralysis  and  occupational  neuritis 164 

Polyneuritis 165 

Particular  clinical  forms 169 

Supplement:  A.  Landry's  paralysis,  paralysis  ascendens  acuta 174 

B.  Leprosy  of  the  nervous  system 175 

II.  The  neuralgias 176 

Supplement:  Herpes  zoster '186 

III.  The  tumors  of  the  peripheral  nervous  system 188 

III 
DISEASES  OF  THE  SPIXAL  CORD 

1.  Normal  and  pathological  physiology  of  the  spinal  cord.     By  Privatdocent  Dr. 

Max  Rothmann,  Berlin 190 

A.  Anatomy  of  the  spinal  cord 190 

B.  Functions  of  the  spinal  cord -  .    .  205 

1.  The  reflex  activity 205 

2.  Conduction  of  motility 221 

3.  Conducting  tracts  for  sensibility 229 

Brown  Sequard  symptom-complex      235 

2.  Systemic  diseases  of  the  spinal  cord 239 

(a)  Tabes  dorsalis.     By  Privatdocent  Dr.  S.  Schoenborn,  Heidelberg  .    .    .  239 

(b)  Friedreich's  disease  (hereditary  spinal  ataxia).    By  Privatdocent  Dr.  S. 
Schoenborn,  Heidelberg 276 

(c)  Spastic  spinal  paralysis.     By  Professor  Dr.  F.  Janim,  Erlangen     .    .    .  282 

(d)  Amyotrophic  lateral  sclerosis.     By  Professor  Dr.  F.  Jamin,  Erlangen   .  292 

(e)  Progressive  spinal  muscular  atrophy  (Duchenne-Aran) .     By  Professor 

Dr.  F.  Jamin,  Erlangen 297 

(f)  Xeurotic   progressive    muscular    atrophy.     By    Professor    F.    Jamin, 
Erlangen 306 

(g)  Subacute   and   chronic   poliomyelitis.     By   Professor   Dr.    F.    Jamin, 

Erlangen 310 

(h)  The  combined  columnar  diseases  of  the  spinal  cord.     By  Professor  Dr. 

F.  Jamin,  Erlangen 313 

(i)    Bulbar  paralytic  diseases.     By  Professor  Dr.  F.  Jamin,  Erlangen  .    .    .  318 


CONTENTS  xi 

Page 

i.  Progressive  bulbar  paralysis 318 

2.  Acute  (apoplectic)  bulbar  paralysis 324 

3.  Pseudo'-bulbar  paralysis.     By  Professor  Dr.  F.  Jamin,  Erlangen  .  333 

3.  Multiple  insular  sclerosis.     By  Professor  Dr.  Hermann  Schlesinger,  Vienna.    .  340 

4.  Syringomyelia.     Spinal  gliosis.     By  Professor  Dr.  Hermann  Schlesinger,  Vienna  350 

5.  Haematomyelia.     By  Professor  Dr.  Hermann  Schlesinger,  Vienna 364 

6.  Focal  diseases  of  the  spinal  cord  due  to  extra-  and  intra-medullary  affections. 

By  Professor  Dr.  R.  Finkelnburg,  Bonn 367 

I.  Traumatic  diseases  of  the  spinal  cord 367 

II.  The  pressure  paralyses  of  the  spinal  cord 373 

(a)  Caries  of  the  spinal  column,  spondylitis  tuberculosa 374 

(b)  Carcinoma  and  other  tumors  of  the  spinal  cord 379 

(c)  Neoplasms  of  the  spinal  cord  and  its  membranes 382 

Tumors  of  the  membranes  of  the  spinal  cord 382 

Intramedullary  tumors 385 

Determination  of  the  location  of  the  tumor    .......  388 

(d)  Pachymeningitis  cervicalis  hypertrophica  (Charcot) 393 

III.  The  syphilitic  diseases  of  the  spinal  cord 394 

Meningomyelitis  chronica  syphilitica 394 

Syphilitic  spinal  paralysis      397 

IV.  Myelitis 400 

Supplement:  Abscess  of  the  spinal  cord 410 

7.  Diseases  of  the  conus  terminalis  and  the  cauda  equina.    By  Dr.  L.  R.  Muller, 

Augsburg 410 

IV 

THE  MYOPATHIES  WITHOUT  DEMONSTRABLE  CHANGES  IN  THE 

NERVOUS  SYSTEM 

By  Oberarzt  Dr.  Hans  Curschmann,  Mayence 

1.  Progressive  muscular  dystrophy 424 

2.  Myotonia  congenita,  Thomsen's  disease 443 

3.  Myasthenia  pseudo-paralytica 437 

4.  Myatonia  congenita 443 

V 

DISEASES  OF  THE  BRAIN 

1.  Normal  and  pathological  physiology  of  the  brain.     By  Professor  Dr.  Hugo 

Liepmann,  Berlin 445 

I.  Introductory  remarks  on  the  anatomy,  physiology  and  pathology  of  the  brain .    .  445 

1.  The  membranes  of  the  brain 445 

2.  Circulation  in  the  brain 446 

Veins  of  the  brain 450 

3.  Morphology  of  the  brain 450 

(a)  Convolutions  and  fissures  of  the  convexity 451 

(b)  The  medial  surface  and  base  of  the  brain 453 

4.  Histological  structure  of  the  cortex 455 

5.  Nuclei  of  the  cerebral  nerves 458 

6.  General  relation  between  structure  and  function  of  the  brain 462 

(a)  Theory  of  projection,  projection  paths  and  projection  fields    .    .  462 


xii  CONTENTS 

Page 

(b)  Mnemic  cortical  fields  and  the  theory  of  association  fields .    .    .    .  467 

(c)  Association  paths ..  471 

(d)  The  commissural  fibres,  especially  the  corpus  callosum    ....  472 

7.  Clinical  localization  in  the  left  hemisphere 474 

8.  Differences  between  the  right  and  left  hemispheres 479 

9.  The  cerebellum 480 

10.  Brain  and  reflexes      482 

11.  Secretory  functions  of  the  brain 485 

II.  Symptomatology  dependent  upon  location 486 

1.  General  symptoms 486 

2.  Projection  disturbances:    Disturbance  in  movement  and  sensation.    .    .  488 

(a)  Motor  symptoms  of  irritation 488 

(/3)  Sensory  symptoms  of  irritation 490 

(A)  Motor  symptoms  of  loss  of  function 491 

1.  Paralyses 491 

2.  Contractures  and  hypertonia 493 

3.  Differences  in  cerebral  paralyses  according  to  the  location  of 

the  injury   .  ■ 496 

(a)  Cortical  foci 496 

(/?)  Capsular  foci 498 

(T)  Focal  injuries  in  the  peduncle  and  pons,  alternating 

paralyses 498 

Volume  of  muscles 499 

(d)  Sensory  symptoms  of  lost  function 500 

(e)  Cerebral  and  cerebellar  ataxia 506 

(f)  Symptoms  of  lost  sensory  function  (disturbances  of  the  special 

senses) 507 

1.  Visual  disturbances,  visual  paths 507 

2.  Auditory  disturbances 515 

3.  Olfactory  and  gustatory  disturbances 518 

3.  Mnemic-associative  disturbances 518 

(a)  Aphasia  disturbances 518 

1.  Introduction 518 

2.  Disturbances  of  the  mnemic-associative  speech  apparatus: 

Aphasia.     Internal  speech 520 

3.  The  speech  regions  in  the  brain 524 

4.  Complete  motor  aphasia 526 

5.  Complete  sensory  aphasia 527 

6.  Total  aphasia 529 

7.  Insular  aphasias ' 530 

8.  Pure  word  dumbness 531 

9.  Pure  word  deafness 533 

10.  Transcortical  aphasias  .    . 534 

Disturbances  in  written  speech 539 

(b)  Agnostic  disturbances 541 

(c)  Apraxic  disturbances 544 

1.  General  remarks 544 

2.  Motor  apraxia 546 

3.  Ideational  apraxia 548 

4.  The  predominance  of  the  left  hemisphere  in  actions  and 

localization  of  apraxic  disturbances 549 


GENERAL  DIAGNOSTICS 

OF 

NERVOUS  DISEASES 
VOL.  I 


I 

GENERAL  DIAGNOSTICS  OF  NERVOUS 
DISEASES 

BY 

S.  SCHOENBORN  (Heidelberg) 


i.  TAKING  THE  HISTORY 

The  dictum  that  medical  art  in  its  clinical  aspect  is  deprived  of  its  best 
foundation  when  lacking  a  good  history  of  the  patient,  sounds  very  old- 
fashioned  to-day,  in  this  age  of  orthodiagraphy  and  precipitin  reactions. 
Should  I  assert,  however,  that  this  dictum  is  nevertheless  correct  and  for  no 
special  field  more  correct  than  for  nervous  diseases,  I  should  not  like  to  be 
summoned,  on  this  ground,  as  star  witness  for  a  supposed  backwardness 
in  neurology.  On  the  contrary,  I  would  state,  that,  rather  it  is  an  advan- 
tage that  within  the  confines  of  our  field  that  endless  splitting  up  into  extreme 
specialism,  recent  and  very  recent,  has  not  as  yet  taken  place,  as  we  see  it  to- 
day in  several  branches  of  internal  medicine.  A  summary  of  our  knowl- 
edge of  neurology,  in  spite  of  the  wealth  of  new  observations  and  facts 
increasing,  too,  year  by  year,  is  still  possible  for  the  individual  physician; 
he  need  not  yet,  because  of  the  confusing  mass  of  separate  sciences,  lose  a 
clear  view  of  the  case  as  a  whole- — that  great  inheritance  of  the  first  neurolo- 
gists and  internists  of  the  last  century.  But  for  this  he  needs,  over  and 
above  the  modern  equipment  of  diagnosis,  before  all,  the  crude  tools  of 
those  masters:  the  history  of  the  patient. 

This  history  of  the  patient  consists  according  to  the  traditional  scheme 
of  anamnesia,  of  status,  diagnosis,  prognosis  and  therapy.  I  find  no  reason 
for  making  any  changes  in  this  scheme.  I  know  of  no  more  comprehen- 
sive one.  The  method  of  "genius  diagnoses"  ("snap"  diagnoses)  at 
first  sight  imposes  only  on  the  novice.  However  certainly  the  experienced 
may  recognize  from  a  distance  a  tabetic  by  his  staggering  gait  and  the  easily 
visible  pupillary  differences  and  a  case  of  Basedow's  disease  by  the  staring 
eyes  and  the  goiter,  he  must  never  rely  on  his  empiricism.  Very  often  exact 
anamnesia  alone  suffices  to  correct  the  would  be  cock-sureness  of  diagnosis 
at  first  sight. 

I  said  that  the  value  of  an  exact  history  of  the  patient  is  especially  high, 
precisely  in  nervous  diseases.  This  holds  true,  to  begin  with,  of  the  anam- 
nesis.    It  is  more  indispensable  here  than  in  most  internal  diseases,  though 


2  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

on  the  other  hand,  it  is  less  often  possible  to  make  the  diagnosis  from  the 
anamnesia  alone,  than  in  these  diseases.  A  sufferer  from  kidney  trouble 
may  by  an  exact  account  of  the  development  of  the  disease,  and  of  his  troubles 
alone,  assist  the  physician  to  a  correct  diagnosis,  and,  on  the  other  hand,  in 
this  case,  the  physician  can  usually  make  the  diagnosis  by  the  examination 
alone,  even  without  any  anamnesia.  Both  are  but  rarely  possible  with  the 
nervous  patient.  He  can  not  off-hand  with  sufficient  exactitude  so  state 
his  troubles  with  respect  to  time  and  place  as  to  make  the  disease  recogniz- 
able by  them  alone,  especially  since,  in  nervous  diseases,  a  slowly  progressing 
course  with  a  large  number  of  slighter  and  more  severe  symptoms  is  the 
rule.  But  almost  without  exception,  not  even  the  most  minute  examination, 
alone,  can  enlighten  as  to  the  genesis  of  the  disease  and  the  troubles  of 
the  patient,  which  are  of  such  great  therapeutic  importance. 

In  taking  up  the  previous  history  we  inquire  first  about  the  family 
history.  Are  there  among  the  ancestors  or  descendants,  mental  diseases, 
characteristic  neuroses,  alcoholism?  Were  the  parents  consanguineous? 
D'id  the  parents  suffer  from  syphilis  probably  or  possibly?  (Mercurial 
treatment,  premature  births.) 

Nor  can  the  unfortunately  all  too  indefinite  question  about  "nervousness 
in  the  family"  be  avoided,  and  it  often  brings  to  light  important  facts. 

All  these  factors  may  be  comprised  under  the  designation  "neuropathic 
taint." 

Then  follow  the  questions  as  to  the  previous  diseases  of  the  patient. 
Here,  one  must  consider  before  all:  normal  birth;  children's  diseases,  espe- 
cially of  the  nervous  kind  (spasms,  infantile  convulsions,  migraine,  pavor 
nocturnus,  enuresis,  etc.),  and  the  thereto  predisposing  disturbances  in  de- 
velopment (rachitis);  infectious  diseases  (measles,  scarlet  fever,  typhoid, 
diphtheria,  influenza);  chronic  use  or  exposure  to  certain  poisons  (alcohol, 
nicotine,  lead,  morphine);  over-exertions  of  every  kind  (mental,  physical, 
especially  too,  sexual);  masturbation.  Particular  attention,  furthermore, 
is  to  be  given  to  two  points:  first,  the  possibility  of  preceding  syphilis,  for 
which  one  should  watch  with  the  greatest  care  and  the  greatest  objectivity 
(chancre,  eruptions,  mercurial  treatment,  symptoms  of  visceral  syphilis, 
miscarriages  and  abortions  in  women;  the  vocation  is  to  be  considered 
also)  secondly,  accidents  or  injuries  experienced,  whereby,  of  course,  in 
the  interest  of  the  patient,  too  clear  a  reference  to  the  traumatism  is,  at  first, 
to  be  especially  avoided  by  the  questioner.  Furthermore,  one  must  inves- 
tigate the  mental  development  (schooling)  and  note  the  vocation  of  the 
patient. 

The  previous  history  and  the  history  of  the  present  trouble  conclude  the 
anamnesis.  While,  in  his  former  questions,  the  investigator  may  proceed 
according  to  a  scheme,  here,  with  minute  consideration  of  all  nervous 
regions,  he  must  often  deviate  from  the  scheme  and  follow  a  definite  idea, 


THE  OBJECTIVE  FINDINGS  3 

which  may  lead  him  in  the  direction  of  fixed  disease  pictures;  for,  as  has 
already  been  mentioned,  the  nervous  patients  scarcely  ever  succeed  in  sen- 
sibly arranging  their  symptoms  in  proper  order  and  even  the  schematic 
questioner  has  before  him,  at  the  end,  usually  a  chaos  of  individual  symp- 
toms, a  disease  history  "without  head  or  tail."  In  particular,  the  physician 
should  attempt  to  ascertain  the  very  beginning  of  the  disease,  its  time  and 
place;  the  correct  diagnosis  often  depends  on  the  temporal  sequence  of  the 
symptoms  (for  instance  spinal  atrophies).  Therefore,  in  suspicion  of  tabes 
one  will  proceed  from  possible  eye  symptoms,  lancinating  pains,  in  a  myeli- 
tis, from  paraesthesias  in  the  feet,  bladder  disturbances,  etc. 

Naturally  the  symptoms  in  the  internal  organs  (stomach,  heart),  must 
be  considered  also. 

It  should,  as  a  rule,  be  possible  to  ascertain  from  the  history  all  symptoms 
of  a  nervous  disease,  that  can  at  all  be  subjectively  perceived. 

2.  THE  OBJECTIVE  FINDINGS 

A.  General  Remarks 

In  the  examination  following  the  history  the  mere  observation  of  the 
nervous  patient  may,  in  itself,  be  of  great  value.     Facial  expression  and  the 
attitude  of  the  body,  especially  in  bed-ridden  patients,  may  give  much  infor- 
mation.    A  rigid  attitude  or  carriage  of  the  body  may  be  dependent  upon 
spastic   pareses    (myelitis,    system-diseases,    central    affections),    paralysis 
agitans,  upon  meningitis,  tetanus,  and  other  affections.     Pains  in  certain 
nervous  regions  often  induce  anomalies  in  the  posture  of  the  body,  which 
mostly  have  in  view  a  relaxation  of  the  nerves  concerned  (sciatica),  or  cause 
involuntary  twitchings  of  the  musculature  in  these  regions  (tic  douloureux). 
Asymmetries,  too,  strike  the  attention  here  at  once,  not  only  those  that  affect 
the  musculature   (cf.  below),  but  also  especially  those  which  affect  the 
structure  of  the  bones.     Scolioses  and  kypho-scolioses  cause  suspicion  of 
the  presence  of  sciatica,  compression  myelitis,  syringomyelia.     Asymmetries 
of  the  face  may  rest  upon  progressive  facial  hemiatrophy,  sympathetic 
affections,  acromegaly.     In  the  latter  case,  we  rind  similar  asymmetries 
in  the  extremities.     Cranial  asymmetry  may  be  due  to  tumors  or  hydro- 
cephalus.    Nor  must  we  overlook  congenital  malformations  (cranial  malfor- 
mation, abnormal  ear  formation,  hare-lip,  polydactylia,  etc.),  but  one  may 
say  that  this  branch  of  neuropathic  taint  has  more  real  importance  in 
psychoses  than  in  nervous  diseases  (probably  most  in  the  so-called  neuroses) . 
There  are  rarely  found  in  nervous  troubles  noticeable  changes  in  the  skin 
and  the  visible  parts  of  the  mucous  membranes.     Here,  again,  traces  of 
syphilis  must  be  most  carefully  followed  (Hutchinson's  triad,  scars  from 
tertiary  processes,   swollen  glands).     Circumscribed,  more  rarely  diffused 
reddening  or  cyanosis,  also  anaemic  pallor,  may  be  interpreted  as  a  sympathetic 


4  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

disease,  occurring  alone,  or  with  spinal  or  cerebral  troubles,  even  with 
neuroses.  Neurogenous  edemas  are  found  in  hysteria,  more  rarely  in 
spinal  diseases.  Ulcerative  changes  we  see  also  in  hysteria  (mostly  through 
self-inflicted  injuries),  likewise  as  severe  trophic  disturbances,  usually  com- 
bined with  synchronous  disturbances  in  sensibility,  especially  in  tabes  and 
syringomyelia,  finally  as  decubital  sores  in  every  long  continued  loss  of  local 
sensation  (also  in'the  mucous  membranes — keratitis  neuroparaly tica) .  Rare 
trophic  neuroses  sui  generis  (Raynaud's  disease)  may  lead  to  ulceration  and 
gangrenous  destructive  processes.  The  blister-like  eruptions  of  herpes 
zoster  belong  to  a  dermatologic-neurologic  borderland;  they  are  combined 
nearly  always  with  neuralgias  in  the  nervous  region  concerned.  We  shall 
have  to  discuss  all  these  points  in  more  detail  later. 

The  physical  methods  of  internal  medicine  in  general  use  are  of  but 
small  service  in  the  examination  of  nervous  patients.  Percussion  may 
occasionally  be  employed  on  the  skull,  in  internal  affections  of  the  cranium; 
a  peculiar  flat  tinny  percussion  note  (reminding  of  the  bruit  de  pot  fele), 
the  so-called  " 'cracked  pot  note'"  is  occasionally  found  on  the  diseased  side 
in  cerebral  tumors.  Palpation  sometimes  serves  for  the  recognition  of  an- 
atomic changes  in  the  peripheral  nerves  and  nerve  sheaths.  It  may  also  be 
important  in  discerning  the  causes  of  a  dysbasia  arteriosclerotica  by  the 
absence  or  the  smallness  or  the  hardness  of  the  peripheral  arterial  pulse. 
By  auscultation,  we  recognize  in  rare  cases  the  presence  of  vascular  tumors 
and  aneurysms  of  the  arteries  inside  the  cranium.  As  an  aside  we  may  re- 
mark that  auscultation  and  percussion  naturally  may  be  of  use  to  us  indirectly 
also  in  neurology,  in  the  recognition  of  the  causes  of  peripheral  paralyses 
(recurrens  paralysis  in  aortic  aneurysms),  etc.,  and  that  the  establishment  of 
sensitiveness  to  pressure  in  bones  and  nerve  trunks  (caries,  neuralgias)  may 
be  of  decisive  importance. 

Great  weight  must  from  the  first  be  laid  upon  the  correct  interpretation 
of  disturbances  of  consciousness  and  the  psyche.  Dimming  of  conscious- 
ness is,  on  the  whole,  easily  recognized;  the  lightest  form  is  usually  desig- 
nated somnolence,  a  condition  of  sleepiness  from  which  the  patient  may  how- 
ever be  awakened  by  a  mere  call,  in  which  correct  answers  can  be  obtained, 
the  next  higher  degree  as  sopor,  with  retained  reflexes,  in  which  awakening 
is  produced  only  by  means  of  violence  (by  stimulation  of  the  skin,  etc.), 
the  highest  degree  as  coma,  with  the  reflexes  lost.  Here  the  patient  can  not 
be  awakened  even  though  force  is  used.  All  three  forms  occur  in  most 
cerebral  diseases  as  well  as  in  all  those  internal  diseases  that  affect  the  cen- 
tral nervous  system. 

Knowledge  of  psychic  disturbances  in  detail  belongs  to  the  realm 
■of  psychiatry,  with  the  fundamentals  of  which,  however,  every  neurologist 
must  be  acquainted.  How  important  are  even  the  lightest  forms  of  these 
disturbances,  often  how  typical !    The  patient's  mood  is  occasionally  charac- 


THE  PSYCHE,  THE  SENSE  ORGANS,  VOICE  AND  SPEECH        5 

teristic  of  his  disease;  the  euphoric  mood  is  seen  mostly  in  multiple  sclero- 
sis, in  Friedreich'' s  disease;  gloomy  morose  psychic  states  in  paralysis  agitans; 
exalted  (maniacal)  moods  in  progressive  paralysis  (which,  as  a  fact,  all 
but  belongs  to  the  purely  psychic  realm).  Stupor,  the  almost  reactionless 
depressive  state  rarely  occurs  in  pure  nervous  diseases;  more  frequently 
the  talkativeness  of  most  neurasthenics  which  must  not  be  at  once  assigned 
to  the  sphere  of  maniacal  conditions.  Especially  typical  is  it  in  traumatic 
neuroses,  in  which,  of  course,  the  exclusion  of  the  patient's  desire  for  lucre 
and  of  conscious  or  unconscious  simulation  is  one  of  the  most  difficult  tasks 
of  the  neurologist. 

In  order  to  have  a  clear  picture  of  the  psyche  of  nervous  sufferers,  it  is 
important  to  win  their  confidence,  to  allow  them  to  talk. 

Then  gradually,  by  clever  inquiries,  the  investigator  may  discover  the 
severe  psychic  symptoms:  delusions,  illusions,  hallucinations.  Disturb- 
ances in  memory,  especially  for  more  recent  events,  are  common  and  impor- 
tant. Intelligence  is  often  considerably  clouded  (dementia,  imbecility, 
idiocy),  sometimes  congenital,  with  especial  frequency  the  result  of  diseases 
during  infancy  (hydrocephalus,  encephalitic  processes)  somewhat  less 
frequently  acquired  at  an  advanced  age  (multiple  sclerosis,  epilepsy,  pro- 
gressive paralysis  of  the  insane,  etc.).  For  many  disturbances  in  memory 
and  intelligence,  the  ability  to  count  is  of  peculiar  importance;  therefore  one 
should  set  the  patient  problems  with  this  in  view.  For  all  psychic  condi- 
tions, finally,  the  writing  of  the  patient  may  be  significant,  which  further- 
more discloses  numerous  disturbances  in  motility  (ataxia,  all  forms  of 
tremor,  writer's  cramp,  etc.). 

All  of  the  lighter  and  some  of  the  more  severe  psychic  disturbances  are 
found  in  the  restricted  field  of  neurology,  if  we  except  progressive  paralysis 
with  the  enormous  variety  of  its  psychic  symptomatic  picture,  in  all  the 
inflammatory  processes  inside  the  cranium,  in  cerebral  tumors,  hydro- 
cephalus, traumatisms  of  all  sorts,  in  multiple  sclerosis,  in  some  "system" 
diseases  (Friedreich's  ataxia),  in  that  strange  combination  of  a  multiple 
neuritis  with  psychic  disease,  known  as  Korsakow's  psychosis,  and  in  numer- 
ous neuroses  (neurasthenia,  hysteria,  epilepsy,  chorea,  paralysis  agitans,  etc.) . 

The  general  examination  of  nervous  patients  in  respect  to  visible  external 
changes,  the  results  of  physical  examination  in  the  narrower  sense  and  of 
that  of  the  psyche,  are  to  be  followed  by  the  examination  of  the  organs  of 
sense. 

B.  Testing  the  Sense  Organs,  Voice  and  Speech 

1.  The  Eye 

Every  test  of  the  function  of  the  organ  of  vision  should  begin  with  an 
examination  of  the  pupils. 


6  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

In  the  normal  state,  both  pupils  are  of  equal  width;  congenital  inequality 
(anisocoria)  occurs  rarely  and  in  contradistinction  to  other  authors  we  do 
not  interpret  it  as  a  sign  of  a  neuropathic  disposition.  In  rare  cases,  this 
inequality  alternates  between  the  two  eyes;  each  eye  in  alternation  with  the 
other  shows  now  a  miosis  (contraction)  now  a  mydriasis  (dilatation).  We 
know  as  little  of  the  origin  of  these  "rapidly  alternating"  pupils  as  we  do 
of  the  so-called  hippiis,  the  rapidly  changing  width  of  the  pupil  of  one  eye 
without  external  stimulation. 

Usually,  however,  pupillary  differences  signify  a  disease  of  the  tracts 
subserving  this  contraction  and  expansion,  at  least  in  one  eye.  Here,  the 
wider  as  well  as  the  narrower  pupil  may  be  the  one  diseased  (no  absolute 
values  exist  for  pupillary  width  in  any  form  that  can  be  used  by  neurologists). 
To  decide  whether  both  eyes  or  which  of  the  two  is  the  one  affected,  we  test 
the  pupillary  movements. 

They  consist  in  a  contraction  of  the  pupils  controlled  by  the  m.  sphincter 
pupillas  (oculomotorius),  which  appears  reflexly,  when  light  falls  upon  the 
eye  and  upon  accommodation,  as  voluntary  concomitant  movement,  with 
convergence  and  energetic  contraction  of  the  orbicularis  oculi,  and  in  a 
dilatation  of  the  pupils  controlled  by  the  m.  dilator  pupillae  (sympathetic), 
which  appears  reflexly  in  the  dark,  and  upon  pain  stimulation  (for  theory 
and  localization  of  pupillary  movements  see  special  section  in  this  text-book) . 

Testing  of  the  reaction  to  light  is  best  carried  out  in  a  darkened  room,  by 
means  of  a  reflector  (forehead-,  eye-mirror)  throwing  concentrated  light, 
coming  from  a  source  behind  the  patient,  into  one  eye.  This  is  the  best 
way  of  establishing  the  normal,  rapid  and  extensive  contraction  of  the 
pupil  under  examination,  the  absence  of  reaction,  the  reflex-pupillary  rigidity 
to  light  {Argyll-Robertson's  phenomenon)  as  well  as  all  the  intermediate 
steps,  the  most  important  of  which  is  the  "sluggish"  reaction,  a  slow  con- 
traction of  the  pupil,  comparable  in  its  course  to  several  other  pathological 
reflexes  (Babinski  toe  reflex) . 

Almost  simultaneously  with  the  process  in  the  illuminated  eye  there 
appears  in  the  non-illuminated,  the  so-called  "consensual"  contraction; 
observation  of  it  may  be  important  for  the  determination  whether  reflex 
disturbances  are  to  be  localized  in  the  centripetal  (opticus)  or  the  centrif- 
ugal (oculomotorius)  reflex  tract,  since  in  the  latter  case,  the  non-illuminated 
pupil  contracts  consensually  in  spite  of  the  areflexia  of  the  illuminated 
pupil,  and  not  in  the  former. 

Theoretically,  sluggish  reaction  and  loss  of  the  reaction  to  light  of  the 
pupil  may  of  course  appear  in  every  disease  that  interrupts  the  reflex  tract 
at  any  point.  Practically,  however,  this  is  the  case  only  in  relatively  few 
well  characterized  diseases  of  the  central  nervous  system,  of  which  the 
metasyphilitic  (tabes,  progressive  paralysis),  meningitis  and  tumor  cerebri 
may  be  mentioned  as  the  most  frequent.     The  rigidity  to  light  may  be  uni- 


THE  EYE,  VISION,  PUPILS  7 

lateral  or  bilateral;  pupils  with  total  rigidity  to  light  are  somewhat  more 
frequently  miotic,  contracted  (often  to  the  size  of  a  "pin  head"),  than 
mydriatic,  dilated. 

Naturally,  testing  the  effect  of  light  falling  upon  the  eye  may — and  this 
will  be  the  rule  in  investigations  as  to  orientation — take  place  in  daylight; 
one  then  has  the  patient  (this  is  the  best  way)  look  through  a  well  lighted 
window  at  a  distant  object  and  fixate  it,  and  covers — this  is  to  be  recom- 
mended in  all  cases — both  eyes  at  the  same  time,  and  then  by  taking  away 
the  covering  (hand,  etc.),  tests  separately  the  direct  and  consensual  light 
reaction  of  each  eye.  But  this  test  may  be  made  exceedingly  difficult  by 
poor  daylight,  very  dark  iris,  or  by  the  light  reflected  from  the  cornea. 

Independently  of  the  pupillary  contraction  upon  entrance  of  light,  con- 
traction upon  accommodation  or  convergence  may  take  place.  Normally, 
both  pupils  energetically  contract  upon  accommodation  of  the  eye  for  near 
objects  (if  the  mm.  interni  are  normal,  with  simultaneous  convergence). 
This  contraction  may  be  retained  in  the  absence  of  the  light  reaction  (fre- 
quently in  tabes)  or  it  may  be  missing  at  the  same  time  as  the  light  reflex 
(tabes,  progressive  paralysis,  cerebral  tumors)  constituting  total  pupillary 
rigidity  (ophthalmoplegia  interna);  a  case  of  isolated  accommodation  pa- 
ralysis occurs  very  rarely  and  is  practically  negligible. 

The  voluntary  contraction  of  the  pupil  upon  innervation  of  the  orbicu- 
laris oculi  is  easily  tested,  but  has  not  yet  attained  pathological  importance. 

Reflex  dilatation  of  the  pupils  is  far  less  important.  We  test  it  by 
shading  the  eye  in  day  light,  as  well  as  by  pain  stimulations  (pinching  or 
pricking  the  skin  of  the  cheeks).  The  undubitably  justifiable  conclusion 
that  an  absence  of  the  dilatation  reflex,  must,  in  general,  point  to  an  affection 
of  the  sympathetic,  can  be  used  but  rarely  neurologically  with  our  slight 
knowledge  of  the  processes  in  the  sympathetic  region.  On  the  other  hand, 
we  find  this  absence  frequently  with  the  synchronous  absence  of  contrac- 
tions to  light  in  the  pupils,  in  tabes,  progressive  paralysis  of  the  insane, 
severe  affections  of  the  substance  of  the  brain;  the  pupils  in  this  case  are 
neither  contracted  nor  dilated  to  a  high  degree,  but  are  of  medium  width. 

Abnormal  miosis  may  depend  upon  absence  of  the  reaction  to  light  as 
well  as  upon  paralysis  of  the  dilator  pupillae,  and  also  (rarely,  almost  only 
in  hysteria)  upon  a  sphincter  spasm.  In  analogous  manner,  we  see  mydri- 
asis in  sphincter  paralysis,  spasm  in  the  dilator  pupillae  and  not  infrequently 
as  a  purely  psychic  reaction  in  terror,  great  mental  exertions,  etc.  Neu- 
rasthenics often  have  permanently  very  dilated  pupils. 

Finally  it  must  be  mentioned  that  in  old  age  all  the  pupillary  reflexes 
usually  decrease,  and  that  certain  poisons  tend  to  dilate  the  pupil  (a tropin, 
hyoscin),  others  to  contract  it  (eserin,  morphine).  In  chloroform  narcosis, 
the  pupils,  as  a  rule,  are  immobile  to  light  and  dilated. 

Next  to  the  test  of  pupillary  reaction,  comes  that  of  the  external  muscles 


8  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

of  the  eye.  Here  are  concerned  the  m.  rectus  ext.  (n.  abducens),  obliquus 
sup.  (n.  trochlearis),  obliquus  inf.,  rectus  int.,  levator  palpebrae  {all  the 
n.  oculomotorius),  while  the  orbicularis  oculi,  supplied  by  the  facialis,  is  to 
be  dealt  with  in  the  test  of  musculature  of  facial  expression. 

Besides  the  action  of  the  m.  levator  palp.,  which  raises  the  upper  eyelid, 
only  the  m.  rectus  ext.  and  the  rectus  int.  are  to  be  tested  in  simple  manner, 
by  asking  the  patient  to  look  energetically  towards  the  right  or  the  left,  or 
better,  with  head  fixed,  to  follow  with  the  eyes,  as  long  as  possible,  an  object 
that  is  moved  in  the  direction  to  the  right  or  to  the  left.  If  one  of  these  two 
muscles  is  hindered  from  acting,  the  eye  concerned  does  not  follow  in  the 
direction  of  its  normal  contraction.  A  second  necessary  consequence  is 
the  appearance  of  double  images  (with  retained  vision),  which,  however,  are 
frequently  consciously  or  unconsciously  suppressed  by  the  patient,  since  he 
fixates  with  one  eye  only.  In  slight  pareses  of  one  muscle,  the  test  for 
double  images  is  often  the  more  delicate  as  contrasted  with  that  for  eye 
movement;  for  this  purpose  one  asks  the  patient  to  say  when  the  object 
which  is  being  slowly  moved  sidewards  is  seen  by  him  double  or  confused, 
and  in  the  former  case,  in  what  relation  the  double  images  stand  to  each 
other.  To  facilitate  the  recognition  of  both  images  (of  which  one  is  usually 
seen  more  brightly,  that  is,  more  clearly),  one  may  place  a  red  glass  before 
one  eye;  the  red  and  the  white  image  can  then,  even  though  partly  overlap- 
ping, be  easily  distinguished  from  each  other.  In  paresis  of  a  rectus  int, 
the  double  images  are  at  the  same  height  and  are  "crossed,"  i.  e.,  the  image 
that  "belongs"  to  the  right  eye  lies  on  the  left  side;  in  paresis  of  the  rectus 
ext.  the  double  images  are  at  the  same  height  and  "uncrossed." 

Examination  of  the  obliqui  and  the  rectus  sup.  and  inf.  is  more  difficult, 
since  their  effects  are  always  somewhat  combined  so  as  to  move  the  eye 
upwards,  downwards,  or  in  a  circle.  The  rectus  inf.  moves  the  eyeball  down- 
wards and  inwards,  the  obliquus  sup.  downwards  and  outwards  and  rotates  it 
somewhat;  the  obliquus  inf.  upwards  and  outwards,  the  rectus  sup.  upwards 
and  inwards,  with  synchronous  rotation.  In  these  muscles,  the  loss  of 
movement  is,  as  a  rule,  very  slight  and  difficult  to  test;  it  is  best  to  examine 
the  double  images,  for  which  the  following  scheme  holds  good : 

In  paralysis  of  the  rectus  inferior,  the  double  images  appear  only  on 
looking  down,  the  images  are  crossed,  inclined  to  each  other,  and  one  below 
the  other,  the  false  image  is  the  lower,  and  upon  lowering  of  the  object 
concerned,  separates  from  the  true  image,  which  is  higher. 

In  paralysis  of  the  obliquus  superior,  double  images  appear  upon  looking 
up;  the  pictures  are  uncrossed,  inclined,  and  one  below  the  other. 

In  paralysis  of  the  obliquus  inferior,  double  images  appear  upon  looking 
up;  they  are  inclined,  one  below  the  other,  but  uncrossed. 

In  paralysis  of  the  rectus  superior,  double  images  appear  upon  looking 
up;  they  are  inclined,  one  above  the  other,  and  are  crossed;  the  false  upper 


THE  OCULAR  MUSCLES  9 

image  leaves  the  true  lower  image  upon  raising  of  the  object  used  in  the 
test. 

Weakness  of  the  eye  muscles  causes  still  other  phenomena  of  functional 
loss,  which,  however,  are  of  less  diagnostic  service  to  the  neurologist.  These 
are  for  every  single  muscle  the  secondary  contracture  of  its  antagonist,  which, 
when  vision  is  turned  directly  forwards,  conditions  an  abnormal  position,  a 
strabismus  (most  clearly  in  paresis  of  the  rectus  int. — strabismus  divergens, 
and  in  paresis  of  the  rectus  ext. — strabismus  convergens);  furthermore, 
occasionally,  the  secondary  deviation  of  the  unaffected  eye,  the  abnormal 
position  of  the  head,  and  finally,  upon  an  attempt  to  exert  the  paralyzed 
muscle,  some  twitchings  of  the  bulbus  in  the  sense  of  the  working  of  this 
muscle  (so-called  paresis-nystagmus,  especially  in  conditions  of  weakness 
in  convalescents). 

Naturally  besides  the  paralysis  of  individual,  and  the  paralyses  of  all 
the  external  muscles  (ophthalmoplegia  externa),  or  of  all  the  eye  muscles 
(total  ophthalmoplegia),  there  occur  also  variously  grouped  paralytic  con- 
ditions in  several  muscles.  Of  these  should  be  mentioned  the  so-called 
conjugate  paralysis  of  the  eye  muscles,  with  loss  of  the  power  to  turn  both 
eyes  laterally  in  the  same  direction  (affecting,  therefore,  only  one  rectus 
int.  and  ext.  of  each  eye) ;  here  the  convergence  of  bulbi  is  usually  retained 
in  spite  of  the  necessary  co-operation  of  the  paralyzed  internus  concerned. 
Then  there  is  convergence  paralysis,  the  counterpart  of  the  disturbance  just 
mentioned,  in  which  both  bulbi  retain  their  ability  to  turn  laterally,  and 
then  the  rare  divergence  paralysis,  which  is  difficult  to  recognize  (the  patient 
can  not  bring  back  to  their  normal  position  the  bulbi  which  have  been  con- 
verged to  look  at  an  object  near  by) . 

Heretofore  we  have  spoken  only  of  the  paralyses  of  the  individual 
muscles.  The  muscular  groups  supplied  by  one  of  the  cerebral  nerves  men- 
tioned, may  also  be  paralyzed  on  one  or  both  sides.  When  the  n.  abducens 
and  the  n.  trochlearis  are  paralyzed,  the  disturbances  may  readily  be  deduced 
from  what  has  already  been  said.  In  paralysis  of  the  oculomotorius,  the  eye 
affected  is,  as  a  rule,  fixated  towards  the  outside  downwards;  and  of  active 
movements,  there  is  possible  only  a  slight  outward  rotation  of  the  bulbus. 
Furthermore,  in  total  oculomotorius  paralysis,  the  pupil  concerned  is  dilated, 
and,  as  a  result  of  paralysis  of  the  levator  palpebrae,  the  upper  eyelid  droops 
{ptosis).  This  ptosis  may  appear  also  as  an  isolated  phenomenon  (in 
hysteria,  occasionally  likewise  in  organic  diseases  as  for  instance  tabes). 
For  the  rest,  there  occur  isolated  and  grouped  paralyses  of  the  eye  muscles 
in  a  great  many  central  and  peripheral  (eye-muscle-nerve)  affections  of  the 
central  nervous  system,  among  which  must  be  mentioned  above  all,  the 
syphilitic  and  metasyphilitic  diseases  (tabes,  progressive  paralysis)  as  well 
as  some  toxic-infectious  processes  (diphtheria). 

As  we  shall  see  later,  in  all  disturbances  of  motility  in  the  human  body, 


io  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

a  second  group  of  disturbances  besides  paralysis  may  be  thought  of,  i.  e., 
hyperkinesis,  spasms,  in  which  we  distinguish  tonic  (continuous  tension  of 
the  diseased  muscle)  and  clonic  spasms  (change  between  tension  and  relaxa- 
tion, twitching  movement).  This  holds  also  for  the  eye  muscles,  in  which, 
however,  spasms  are  very  much  rarer  than  paralyses.  Thus  a  strabismus 
based  upon  genuine  spasm  (usually  convergent)  is  very  rare,  observed  almost 
exclusively  in  hysteria.  Let  us  mention  here  that  by  far  the  greatest  number 
of  all  cases  of  strabismus  (so-called  muscular  or  concomitant  squint)  are 
not  based  upon  a  muscular  paralysis,  but,  so  to  say,  only  upon  conditions 
of  weakness,  a  so-called  disturbance  in  the  equilibrium  of  the  muscles;  that 
in  spite  of  the  changed  position  of  the  bulbi,  it  proceeds  usually  without 
double  vision  and  disturbances  in  motility,  and  that  as  a  result,  the  typical 
test  for  the  individual  eye  muscles  gives  a  negative  result.  Cf .  on  this  point 
text-books  on  ophthalmology.  We  must  sometimes  regard  as  tonic  spasm, 
the  remarkable  phenomenon  of  conjugate  deviation,  in  which  both  eyes  are 
held  fixed  in  a  position  of  rotation  towards  the  right  or  the  left  (especially 
in  cerebral  tumor  in  certain  regions,  often  accompanied  with  rotation  of 
the  head  to  the  same  side).  In  rarer  cases,  it  may  rest  also  upon  a  paralysis 
of  the  antagonists. 

The  various  forms  of  nystagmus  may  be  interpreted  as  clonic  spasms 
of  the  eye  muscle:  short  twitchings  following  one  another  in  slow  or  rapid 
succession,  of  one  or  both  bulbi  in  a  lateral  direction  (nystagmus  horizon- 
talis),  in  a  vertical  direction  (N.  verticalis),  or  in  rotation  (N.  rotatorius). 
We  find  it  (cf.  above  )  frequently  in  pareses  of  individual  muscles  in  respect 
to  and  in  the  direction  of  their  activity  (especially  N.  horizontalis,  which, 
moreover,  in  "end  positions"  of  the  bulbi,  in  the  extreme  lateral  direction, 
are  not  infrequently  found  in  the  healthy,  too),  furthermore,  congenital 
(here  often  very  intensive,  when  vision  is  directed  straight  forward),  as 
vocational  disease  (miners),  in  blindness  and  finally — this  is  of  especial 
importance  for  the  neurologist — in  a  series  of  affections  of  the  brain,  above 
all  in  multiple  (cerebro-spinal)  sclerosis. 

While  testing  the  individual  eye  muscles  is  one  of  the  most  difficult 
chapters  in  the  diagnostics  of  nervous  diseases  and  now  and  then  makes 
.assistance  from  an  ophthalmologist  desirable,  examination  of  the  back- 
ground of  the  eye  should,  in  its  main  points,  be  well  mastered  by  the  neurolo- 
gist. We  may  exclude  here  the  real  affections  of  the  retina  and  chorioidea, 
which  less  frequently  occupy  the  attention  of  the  neurologist  (at  most  occa- 
sionally as  sign  of  neuropathic  disposition,  as  in  retinitis  pigmentosa  and 
other  congenital  diseases),  but  must  consider  that  sometimes  the  diagnosis 
of  a  cerebral  disease  seems  to  be  erroneously  confirmed  by  an  affection  of  the 
retina,  as  in  a  retinitis  albuminurica  confined  to  the  disc  and  simulating  a 
neuritis  optica. 

Two  ophthalmoscopic  findings  especially  are  of  interest  to  us : 


OPTIC  NEURITIS  AND  ATROPHY  n 

Neuritis  optica,  or  choked  disc  and  atrophy  of  the  optic  nerve. 

In  neuritis  optica  the  disc  appears  clouded,  reddened,  or  grayish  red  in 
color,  the  veins  are  dilated,  the  arteries  of  the  disc  contracted,  the  edge  of  the 
disc  not  clearly  marked.  The  diameter  may  be  considerably  increased. 
In  pronounced  choked  disc,  there  is,  besides,  an  ophthalmoscopic,  usually 
easily  recognizable  protrusion  of  the  disc;  the  blood  vessels  appear  to  be 
snapped  off  at  the  edge  of  the  disc,  and  disappear  in  the  diffuse  redness  of 
the  enlarged  disc. 

Concerning  differentiation  between  neuritis  optica  (papillitis)  and  choked 
disc,  it  is  all  the  more  difficult,  considering  what  has  been  said,  inasmuch 
as  there  is  by  no  means  a  consensus  of  opinion  as  to  the  cause  of  the  disease. 
A  mechanical  congestion  (cerebral  tumor)  through  increase  in  the  intra- 
cranial pressure  (accumulation  of  large  quantities  of  cerebro-spinal  fluid 
inside  the  cranium)  and  a  gathering  of  fluid  in  the  sheath  of  the  optic  nerve 
may  give  rise  to  ''papillitis"  as  well  as  choked  disc;  on  the  other  hand,  toxic 
products  in  infectious  and  other  diseases  may  evoke  choked  disc  also  in 
addition  to  the  neuritis  optica,  or  papillitis  which  usually  results.  In  general, 
the  ophthalmologist  will  incline  rather  to  a  differentiation  of  the  two  condi- 
tions, the  neurologist  to  the  identification  of  them,  with  the  restriction  that 
they  both  represent  different  degrees  of  the  same  affection. 

The  acuteness  of  vision  in  neuritis  optica  is  nearly  always  normal  in  the 
beginning  of  the  disease,  and  very  frequently  later  also,  at  least  central 
vision,  whereas  contractions  of  the  visual  field  (cf .  below)  frequently  appear 
later;  but  the  beginner  must  never  forget  that  there  may  exist  even  a  high 
degree  of  choked  disc  without  the  knowledge  of  the  patient.  Thus  the 
ophthalmoscopic  picture  here  by  no  means  corresponds  to  the  degree  of 
the  visual  disturbance. 

The  recognition  of  choked  disc  is  in  nearly  all  cases  easy;  only  the 
lightest  degrees  of  incipient  papillitis  may  cause  difficulties;  of  course  one 
must  not  be  deceived  by  the  apparent  indistinctness  of  the  edges  of  the 
disc  in  refraction  anomalies. 

Naturally  we  find  choked  disc  most  frequently  in  processes  which  cause 
congestion,  especially  in  tumor  of  the  brain.  Here  it  is  usually  (but  by  no 
means  always)  bilateral.  The  situation  and  size  of  the  tumor  are  not  always 
decisive  as  to  the  origin  and  intensity  of  choked  disc,  still,  very  large  tumors 
can  scarcely  run  their  course  without  accompanying  papillitis,  while,  on 
the  other  hand,  we  must  remember  that  frequently  in  recent  times  there 
have  been  observed  cases  of  typical  tumor  symptoms  with  choked  disc, 
though  in  the  operation  or  the  autopsy,  no  tumor  was  found  (Nonne). 
Meningitis  of  any  kind  may  cause  papillitis;  it  is  found  with  special  frequency 
in  meningitis  serosa  and  in  hydrocephalus.  In  all  forms  of  cerebro-spinal 
syphilis,  in  lead  poisoning,  in  acute  infectious  diseases,  in  chlorosis,  it  occa- 
sionally occurs;  a  rheumatic  inflammation  of  the  opticus,  comparable  to 


12  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

"rheumatic"  paralysis  of  the  eye  muscles  is  also  observed.  On  the  other 
hand,  it  is  rarely  or  never  found  in  encephalitis,  nor  in  apoplexies  of  any 
kind,  and  it  is  lacking  almost  without  exception  in  two  diseases  which  are 
accompanied  by  very  typical  changes  in  the  background  of  the  eye,  that  is, 
in  tabes  and  in  multiple  sclerosis.  In  both  cases,  as  a  rule,  we  find  in  its 
stead  atrophy  of  the  optic  nerves. 

Atrophy  of  the  optic  nerve  is  ophthalmoscopically  recognizable  by 
the  abnormal  pallor  of  the  disc,  which  may  lead  to  a  porcelain  white  colora- 
tion or  discoloration,  but  which  may  also  present  only  a  slightly  paler  color 
in  one-half  of  the  disc.  In  the  latter  case,  diagnosis  is  nearly  always  difficult, 
since,  as  is  well  known,  there  are  present  in  normal  conditions  differences  in 
clearness  in  the  separate  parts  of  the  disc,  and  especially  the  part  first  affected, 
the  temporal  half  of  the  disc  is  mostly  subject  to  these  fluctuations.  In 
these  incipient  cases,  there  is  lacking,  too,  that  sharp  almost  hard  outline  of 
the  white  disc  against  the  red  retina,  that  is  so  characteristic  of  the  final 
stage.  The  width  of  the  vessels  is  usually  not  changed  to  any  considerable 
extent. 

Atrophy  of  the  optic  nerve  may  appear  as  a  primary  phenomenon,  and  it 
may  also  represent  the  final  stage  of  a  neuritis  optica,  that  is,  be  a  secondary 
phenomenon.  The  latter,  which  may  occur  in  all  the  diseases  men- 
tioned above,  is  usually  accompanied  by  considerable  disturbance  in  vision 
(especially  loss  in  the  visual  field) ,  whereas  primary  atrophy  may  exist  for  a 
long  time  without  any  subjective  disturbance,  and  is  therefore  often  over- 
looked (sometimes,  especially  in  multiple  sclerosis,  there  occur  also  sudden 
transitory  visual  disturbances  of  high  degree,  though  the  background  of  the 
eye  shows  nothing  else  but  the  always  equally  slight  "temporal  paling"  of 
the  disc).  Except  when  it  follows  traumatism,  opticus  atrophy  is  nearly 
always  bilateral,  though  often  developed  in  different  degree  in  the  two  eyes. 
Primary  atrophy  of  the  optic  nerves  is  found  almost  only  in  tabes,  progress- 
ive paralysis  of  the  insane,  and  multiple  sclerosis;  in  the  former  case  it  is 
usually  total  and  progressive;  in  the  latter  partial  and  more  or  less  stationary. 
Whether  as  a  matter  of  fact,  it  is  not  frequently  secondary  in  multiple  scle- 
rosis, arising  after  a  neuritis  belonging  to  the  prodromal  stage  so  often  mis- 
taken for  a  "hysteria,"  must,  owing  to  the  scarce  findings  from  examination 
at  this  prodromal  stage,  remain  undecided. 

Cases  of  so-called  neuritis  retrobulbaris  occur  rarely  in  the  practice  of  the 
neurologist,  but  more  frequently  in  that  of  the  ophthalmologist.  Ophthal- 
moscopically, they  are  recognizable  not  at  all,  or  by  an  (secondary)  atrophy 
of  the  papilla  of  moderate  degree;  on  the  other  hand,  they  show  the  typical 
visual  disturbance  of  a  central  loss  in  the  visual  field  for  colors  (red  and 
green).  Retrobulbar  neuritis  is  usually  toxic,  or  toxic-infectious,  and 
occurs  as  an  isolated  phenomenon  or  with  a  polyneuritis  which  owes  its 
origin  to  the  same  causes.     The  most  common  causes  are  chronic  poisoning 


THE   VISUAL  FIELDS  13 

with  alcohol  or  nicotine,  also,  diphtheria.  A  medicinal  neuritis  retrobul- 
baris  occurs  also.  Frequently  retrobulbar  neuritis  optica  has  been  observed 
as  a  very  early  prodromal  symptom  of  multiple  sclerosis  {Uhthoff,  and  others). 

The  real  examination  of  visual  disturbances  in  nervous  diseases — test  of 
acuteness  of  vision — is,  in  the  main,  the  task  of  the  oculist.  But  neverthe- 
less, it  is  evident  that  no  neurologist  will  fail  to  attempt  an  approximate 
determination  with  the  assistance  of  Snellen's  types,  of  counting  fingers  at  a 
certain  distance,  etc.  An  examination  of  the  visual  field,  which  is  generally 
easily  feasible,  is  earnestly  recommended.  While  one  covers  one  of  the 
patient's  eyes,  and  asks  him  to  fixate  a  point  with  the  other,  at  the  distance 
of  from  one-half  to  one  meter,  small  pieces  of  white  or  colored  paper  are 
introduced  into  the  visual  field  area,  and  one  moves  the  pieces  of  paper  from 
the  outer  edge  of  the  visual  field  (that  is,  behind  or  above  or  near  the  patient's 
head)  slowly  in  the  direction  of  the  point  fixed  by  the  patient  up  to  that  point 
itself,  and  then  tells  the  patient  to  say  when  he  catches  sight  of  them  first 
and  whether  they  disappear  again  on  the  way  (central  scotoma).  For 
practical  reasons,  one  chooses  as  the  main  direction  of  these  movements, 
first  the  main  meridians,  above  and  below,  outside  and  inside,  then  even- 
tually an  intermediate  meridian.  All  this  can  be  done  far  more  easily  with 
the  various  perimeters,  some  of  which  are  quite  cheap,  on  which  one  can 
read  the  degrees  directly  (degrees  in  width  of  the  approximately  half  spheri- 
cal field  of  vision) .  One  must  consider  that  the  visual  field  in  normal  con- 
dition is  variously  extended  (for  white  above,  500  to  6o°;  below,  6o°  to  700; 
outside,  900;  inside,  6o°;  for  colors,  considerably  less)  and  that  individual 
differences  occur  also.  One  must  never,  especially  when  using  a  perimeter, 
fail  to  enter  the  results  upon  printed  diagrams  of  the  visual  field,  as  this  is 
the  only  way  to  get  an  accurate  picture  of  the  visual  field. 

Disturbances  in  the  visual  field  occur  with  and  without  other  visual 
disturbances,  and  with  or  without  changes  in  the  background  of  the  eye. 
We  differentiate  the  concentric  contraction,  the  peripheral  scotoma,  the 
central  scotoma  and  hemianopsia.  The  concentric  contraction,  a  diminution 
on  all  sides  (for  white  and  colors,  or  only  for  colors)  of  the  visual  field,  as 
well  as  scotoma,  the  loss  in  spots,  which,  according  to  the  location  of  the  spot 
in  the  visual  field,  is  designated  central  (near  the  point  of  fixation)  or  periph- 
eral, we  find,  as  a  rule,  in  neuritis  optica  (papillitis  or  neuritis  retrobulbaris) 
or  in  atrophy  of  the  optic  nerve.  In  contraction,  the  disease  of  the  nerves  is 
more  frequently  diffuse,  in  scotoma  it  strikes  only  some  bundles  of  fibres. 
Therefore  we  find  both  disturbances  in  tabes,  multiple  sclerosis,  polyneuritis, 
in  the  processes  above  mentioned  which  lead  to  choked  disc,  and,  finally, 
isolated  also;  the  concentric  contraction  is  frequent  too  in  hysteria. 

Hemianopsia,  the  loss  of  an  entire  half  of  the  visual  field  is  less  frequent. 
Here,  it  is  a  question  nearly  always  of  the  loss  of  the  outer  or  inner  (not  the 
upper  or  lower)  half,  and,  moreover,  preponderantly  in  both  eyes  at  once, 


i4  GEXERAL  DIAGXOSTICS  OF  NERVOUS  DISEASES 

corresponding  to  the  partial  crossing  of  the  fibres  of  the  optic  nerves.  One 
speaks  of  homonymous  (bilateral)  hemianopsia,  if  both  right  or  both  left 
halves  are  lost;  on  the  other  hand,  of  bitemporal  hemianopsia  {heteronymous 
hemianopsia),  if  both  external  halves  of  the  visual  field  are  missing.  A 
binasal  hemianopsia  occurs  virtually  never.  Hemianopsia  is  easily  recog- 
nized by  the  test  suggested  for  the  visual  field. 

Hemianopsia  is  found  in  lesions  of  the  chiasma  and  of  the  tractus  opticus; 
and,  moreover,  the  latter  produces  homonymous  hemianopsia,  and,  on  the 
other  hand,  the  lesion  of  the  chiasma  (at  least  of  a  middle  portion  containing 
the  crossing  of  the  fibres  of  the  optic  nerve)  leads  to  bitemporal  hemianopsia. 
Diseases  of  the  chiasma  are,  as  a  rule,  evoked  by  small  tumors  at  the  base 
of  the  brain,  while  the  tractus  may  naturally  be  affected  by  every  agent  that 
injures  the  substance  of  the  brain.  Especially  important  are  the  cases  in 
which  a  homonymous  bilateral  hemianopsia  presents  the  only  sign  of  a 
lesion  (hemorrhage,  tumor)  of  the  occipital  lobe,  in  which  the  visual  center 
is  situated. 

Hemianopsia,  in  contrast  to  concentric  contraction,  is  very  clearly 
present  in  the  consciousness  of  the  patient,  since  it  is  frequently  combined 
with  other  disturbances,  concentric  contraction  of  the  field  of  vision,  visual 
hallucinations,  etc.  Theoretically,  moreover,  every  hemianopsia  belonging 
to  the  tractus  opticus  should  exhibit  an  objective  symptom;  there  should  be 
no  pupillary  reflex  to  light  thrown  upon  the  eye,  if  one  illuminates  only  the 
"blind"  half  of  the  retina  (hemianopic  pupillary  rigidity).  This  does 
occur  occasionally,  but  is  often  difficult  to  demonstrate. 

Monocular  diplopia,  double  vision  in  one  eye  alone,  an  abnormality 
occurring  almost  exclusively  in  hysteria,  does  not  belong  to  the  real  organ- 
ically conditioned  disturbances  of  the  apparatus  of  vision;  nor  does  exoph- 
thalmus,  the  protrusion  of  one  or  both  eyes.  We  find  this  phenomenon 
unilaterally  in  tumors  of  the  orbit  or — through  congestion — also  in  tumors 
of  the  interior  of  the  cranium;  bilaterally,  above  all,  in  Basedow's  disease. 
In  Basedow's  disease  the  origin  of  the  symptom  is  not  altogether  clear;  but 
it  probably  depends  upon  a  swelling  of  the  retrobulbar  tissue,  such  as  occurs, 
and  sometimes  periodically  under  other  conditions. 

2.  Hearing 

While  examination  of  the  eyes  has  proved  to  be  very  useful,  strangely 
enough,  the  value  of  the  ear  findings  has  so  far  proved  to  be  comparatively 
slight  for  neurological  diagnosis.  In  part  this  is  due  to  the  fact  that  genuine 
nervous  diseases  of  the  ear  are  really  rare;  in  part  also  to  the  difficulty  of 
diagnosis  in  these  affections.  Only  very  recently  have  clinicians  (Witt- 
maack)  begun  to  look  with  more  interest  upon  the  diseases  of  the  inner  ear. 

Three  groups  of  symptoms  are  of  use  in  neurologic — otiatric  diagnostics; 


HEARING  15 

subjective  auditory  disturbances  (difficulty  in  hearing,  hyperacusis,  tinnitus 
aurium,  etc.),  objective  changes  in  the  ability  to  hear  (Weber's  and  Rinne's 
experiments)  vertigo,  nystagmus,  disturbances  in  equilibrium.  All  the 
other  resources  of  the  otologist,  especially  the  whole  of  otoscopy,  may  at 
most  be  used  indirectly  by  the  neurologist,  for  the  entire  group  of  disturbances 
in  the  sound  conducting  apparatus  does  not  allow  any  conclusions  to  be 
drawn,  as  to  the  presence  of  a  nervous  disease,  excepting  only  anomalies, 
like  the  occasional  occurrence  of  laxness  of  the  ear  drum  through  atrophy 
of  the  m.  tensor  tympani  in  advanced  dystrophy. 

Of  the  subjective  auditory  disturbances ,  it  is  most  difficult  to  use  tinnitus 
aurium,  ringing  or  buzzing  of  the  ears,  etc.,  for  local  diagnosis.  This 
occurs,  it  is  true,  especially  in  affections  of  the  inner  ear,  which,  however, 
may  be  of  most  varying  types:  arterio-sclerosis,  neuritis  acustica,  transitory 
disturbances,  usually  of  unknown  origin,  such  as  tinnitus  aurium  of 
chlorotics,  the  ringing  in  the  ears  which  appears  paroxysmally  in  Meniere's 
disease,  etc.  Hyperacusis  (oxyecoia)  is  found  in  paralysis  of  the  facial  nerve 
(cf .  above) ,  and  in  some  neuroses,  especially  in  hysteria.  Every  degree  of  dif- 
ficulty in  hearing  occurs  in  diseases  of  the  inner  ear,  in  those  that  are  really 
"nervous"  (labyrinth  and  cochlea)  as  well  as  in  those  of  the  sound  conduct- 
ing apparatus  (tube,  tympanic  cavity,  drum,  auditory  meatus),  and  does  not, 
upon  superficial  examination  permit  of  differential  diagnosis  between  these 
two  groups.  This  first  test  of  the  ability  to  hear  is  made  as  follows:  after 
telling  the  patient  to  close  one  of  his  ears,  words  or  numbers  are  whispered 
or  he  is  allowed  to  hear  the  ticking  of  a  watch,  and  the  limit  of  audition  is 
measured  in  meters  (from  the  ear).  Rinne's  and  Weber's  experiments 
afford  a  more  delicate  test,  in  as  much  as  both  are  concerned  with  the  con- 
ductivity of  the  bones  of  the  head. 

(a)  Rinne's  Experiment. — The  handle  of  a  tuning  fork  pitched  at  low 
c,  after  being  set  in  vibration  is  placed  upon  the  mastoid  process  of  the 
affected  ear.  In  normal  cases,  when  the  sound  is  no  longer  audible  to  the 
patient  by  this  sort  of  "bone  conduction"  perception  is  still  possible 
through  "air  conduction,"  if  one  holds  the  fork  close  to  the  ear  (Rinne's 
positive  test").  The  case  is  the  same — usually  with  diminished  intensity 
of  perception — if  there  be  present  difficulty  in  hearing  of  nervous,  labyrin- 
thine type.  If,  on  the  other  hand,  Rinne's  test  is  "negative,"  that  is,  if 
the  sound  be  heard  longer  by  means  of  the  bones  than  through  conduction 
by  air,  which  occurs  only  in  difficulty  in  hearing — this  defect  must  be 
traced  back  to  the  sound  conducting  apparatus. 

ib)  Weber's  Experiment. — A  vibrating  tuning  fork  is  placed  upon  the 
middle  of  the  frontal  or  occipital  aspect  of  the  head.  In  normal  conditions 
the  sound  is  heard  by  each  ear  with  equal  intensity.  Should,  however,  a 
unilateral  disturbance  in  hearing  obtain — and  the  experiment  is  valuable 
only  in  such  conditions — and  the  sound  be  more  perceptible  by  the  better 


16  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

hearing  ear,  then  the  disturbance  lies  in  the  reception  (nervous)  apparatus 
of  the  other,  namely  the  affected  ear.  But  should  the  patient  localize  the 
sound  in  the  less  perceptive  ear  then  the  disturbance  lies  in  the  sound  con- 
duction apparatus  of  the  affected  ear. 

We  must  mention,  furthermore,  that  in  diseases  of  the  inner  ear,  as  a 
rule,  the  distinctness  of  audition  is  impaired  especially  in  regard  to  the 
higher  tones. 

Disturbances  in  equilibrium,  nystagmus  and  vertigo,  in  so  far  as 
they  proceed  from  the  ear,  are  probably  always  to  be  traced  back  to  the  inner 
ear,  the  nervous  apparatus  (also  indirectly  in  diseases  of  the  tympanic  cavity) . 
The  former  occur  spontaneously  especially  in  the  peculiar  attacks  that  were 
formerly  supposed  to  be  characteristic  of  the  general  concept:  "Meniere's 
disease,"  and  besides  this,  may  easily  be  evoked  in  pathological  cases 
(galvanization  with  weak  currents).  A  feeling  of  dizziness  (ear  vertigo), 
occurring  in  attacks  as  well  as  continuously,  is  very  rarely  lacking  in  disease 
of  the  inner  ear.  A  real  nystagmus  is,  as  a  rule,  evoked  in  labyrinthine 
affections,  upon  turning  the  axis  of  the  body.  According  to  the  most  recent 
views,  these  symptoms  also  make  possible  a  differential  diagnosis  between 
disease  of  the  n.  vestibularis,  or  of  the  labyrinth,  and  of  the  n.  cochlearis, 
or  of  the  cochlea  and  the  organ  of  Corti;  to  the  n.  vestibularis  belong  dis- 
turbance in  equilibrium  and  the  feeling  of  dizziness,  to  the  cochlearis,  the 
nervous  auditory  disturbance,  in  a  stricter  sense. 

"Nervous"  auditory  disturbances  are  found  in  fracture  of  the  base  of 
the  skull,  tumors  of  the  base,  arterio-sclerosis,  in  the  changes  due  to  old 
age,  and  known  as  "otosclerosis,"  in  multiple  sclerosis,  in  tabes  dorsalis 
(as  genuine  degeneration  of  the  nerves,  comparable  to  atrophy  of  the  optic 
nerve),  in  isolated  neuritis,  or  in  neuritis  complicated  with  polyneuritis 
(Frankl-Hochwart) ,  hysteria,  neurasthenia,  and  especially  in  traumatic 
neurasthenia  (the  favorite  hypothesis  of  "labyrinthine  concussion"). 

3.  Taste  and  Smell 

The  sense  of  smell  is  of  least  neurological  usefulness,  in  spite  of  its  so  fre- 
quent disturbances  in  daily  life;  disorders  are  mostly  dependent  upon  catarrhs 
of  the  mucous  membranes,  not  upon  diseases  of  the  olfactory  nerves  them- 
selves. Above  all,  therefore,  one  must  establish  in  neurological  investiga- 
tion whether  the  olfactory  pathways  are  clear,  and  no  catarrh  is  present. 
If  everything  is  well  in  both  these  respects,  one  must  test  the  acuteness  of 
the  sense  of  smell  (naturally  for  each  nostril  separately)  by  holding  odorous 
substances  in  front  of  the  nose,  avoiding  of  course  those  which  stimulate 
the  sensory  end  organs  of  the  trigeminus  (ammonia).  As  a  rule,  I  use 
asafoetida,  tincture  of  valerian,  oil  of  cloves  and  oil  of  roses  (or  eau  de  Co- 
logne).    A  gradation  of  the  acuteness  of  the  sense  of  smell  is  all  the  less 


TASTE  AXD  SMELL  17 

possible  for  the  various  odors,  since  the  delicacy  of  the  sense  of  smell,  the 
"good  nose,"  varies  exceedingly  in  normal  conditions  and  in  a  large  number 
of  healthy  persons  is  developed  very  scantily.  The  odorous  substances 
mentioned,  should,  as  a  rule,  be  recognized. 

In  hysteria  there  occur  hyperosmia  (hyper-sensitiveness)  and  anosmia 
(lack  in  sensitiveness  to  olfactory  stimuli).  In  tumors  of  the  base  of  the 
cranium,  of  the  ethmoid  bone,  etc.,  we  find  occasionally  unilateral  lowering 
of  the  ability  to  smell,  likewise,  in  meningitis  and  fractures. 

Somewhat  more  useful  is  the  test  of  the  sense  of  taste.  A  prior  condition, 
however,  is  that  the  sense  of  smell  be  not  simultaneously  disturbed  (catarrh 
in  the  head) .  We  conduct  the  test  as  follows :  with  a  little  glass  rod  we  place 
in  succession  a  drop  each  of  solutions  representing  the  four  main  qualities 
of  taste,  sweet,  sour,  salt,  bitter — sugar,  vinegar,  salt,  quinine — on  the  out- 
stretched tongue  in  this  manner: 

(a)  on  the  anterior  half  of  the  tongue,  separately  on  the  right  and  left 
sides ; 

(b)  on  the  posterior  half  of  the  tongue,  separately  on  the  right  and  left 
sides. 

After  applying  each  drop  of  the  solution  one  waits  a  few  seconds,  then 
wipes  off  the  tongue  energetically  with  a  dry  cloth,  has  the  patient  withdraw 
his  tongue  and  state  the  taste  concerned.  We  do  this  for  the  following 
reasons :  In  respect  to  the  sense  of  taste,  the  anterior  half  of  the  tongue  is 
under  the  dominion  of  the  chorda  tympani,  that  is,  of  the  n.  lingualis;  the 
posterior  half  and  the  palate  under  that  of  the  n.  glossopharyngeus.  There- 
fore the  test  is  made  separately.  The  tongue  is  drawn  back  because  many 
patients  have  no  taste  sensations  upon  the  extended  tongue.  In  general  sour 
is  better  recognized  on  the  tip  of  the  tongue,  bitter  on  the  posterior  half  of 
the  tongue. 

Disturbances  in  taste  come  to  be  diagnostically  important  almost  only 
in  diseases  of  the  n.  facialis  for  clearer  localization  (chorda  tympani),  also, 
however,  in  disturbances  of  the  n.  trigeminus  III.  The  diseases  of  the 
glossopharyngeus  which  are  rare  in  themselves,  are  concerned  only  excep- 
tionally. Progressive  paralysis  and  neurasthenia  (especially  their  depress- 
ive forms)  are  frequently  accompanied  by  disturbances  in  taste,  which  in 
that  case,  however,  affect  the  entire  tongue. 

4.  Disturbances  in  Voice  and  Speech 

These  processes  belonging  to  the  realm  of  the  cerebral  nerves,  if  not  to 
that  of  the  actual  nerves  of  sense,  may  be  briefly  discussed  here. 

Disturbances  in  voice  occur  mainly  in  two  forms:  as  voicelessness (aphonia) 
and  as  hoarseness;  abnormally  high  pitch  of  the  voice  (squeaking  voice, 
etc.),  is  rare,  occurring  almost  exclusively  in  hysteria.     Aphonia  also,  in  the 


i8 


GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 


great  majority  of  cases  in  which  it  comes  to  the  notice  of  the  neurologist,  is 
of  hysteric,  that  is,  of  psychogenous,  origin,  but  even  detection  of  other 
stigmata,  etc.,  must  not,  as  occasionally  happens,  deter  the  investigator  from 
Iaryngoscopic  examination.  For  even  if  it  occurs  rather  frequently  that  a 
hysteric  aphonia  is  treated  for  weeks  as  chronic  laryngitis,  still,  a  patient 
suffering  from  catarrh  has  sometimes  been  branded  as  hysteric — the  worse 


Fig.  i  — Bilateral  paralysis  of  the 
posticus  in  the  moment  of  inspiration. 
{After  Ziemssen.) 


Fig.    2. — Paralysis  of  both  N.  thyreoary- 
taenoidei  interni.     {After  Ziemssen.) 


Fig.  3. — Paralysis  of  the  recurrens  at  the  left  side  (position  during  inspiration).     {After  Ziemssen.) 


Fig.  4. — Paralysis  of  the  arytaenoi- 
deus.     {After  Ziemssen.) 


Fig.  5. — Bilateral  paralysis  of  the  thy- 
reoarytaenoidei  with  synchronous  paresis 
of  the  arytaenoideus.     {After  Ziemssen.) 


fate!  The  Iaryngoscopic  examination  must  therefore  be  as  familiar  to  the 
neurologist  as  the  ophthalmoscopic.  The  technique  and  the  details  of  the 
Iaryngoscopic  findings  do  not  belong  here,  but  the  disturbances  first  men- 
tioned (aphonia,  hoarseness)  are  nearly  always  the  consequences  of  a  lesion 
of  the  n.  laryngeus  inf.  (recurrens),  which  according  to  its  severity,  presents 
very  different  Iaryngoscopic  pictures.  Of  these  we  shall  mention  the  three 
most  important. 


DISTURBANCES  IN  VOICE  AND  SPEECH  19 

1.  In  total  recurrens  paralysis,  the  vocal  cord  concerned  (or  in  bilateral 
paralysis,  both  vocal  cords)  remains  during  phonation  and  inspiration  in 
the  same  immovable  position  between  the  ad-  and  abduction  (cadaver 
position) . 

2.  In  so-called  internus  paralysis  (paralysis  of  the  thyreo-arytaenoidei 
interni)  the  vocal  cords  are  not  altogether  closed  in  phonation,  but  leave  an 
oval  slit  open;  they  remain  lax,  and  consequently  hoarseness  results. 

3.  In  so-called  posticus  paralysis  (paralysis  of  the  cricoarytenoids 
postici)  phonation  is  as  a  rule  normal,  but  there  usually  appears  inspiratory 
dyspnoea,  since  the  muscles  mentioned  are  dilators  of  the  glottis,  and  there- 
fore upon  their  loss,  the  vocal  cords  are  brought  pathologically  nearer  to 
each  other,  are  drawn  in.  In  unilateral  paralysis  there  is  only  inspiratory 
stridor;  bilateral  posticus  paresis  may,  especially  upon  exertion  and  the 
resulting  forced  breathing,  lead  to  severe  dyspnoea  (Figs.  1  to  5). 

All  these  conditions — there  are  in  addition  a  number  of  rarer  laryngeal 
pareses,  all  of  which  cannot  be  mentioned  here — are  found  in  recurrens, 
or  in  vagus  affections,  which  localized  peripherally  more  frequently  than 
centrally,  may  be  evoked  through  tumors,  traumatisms,  aortic  aneurysms, 
etc.  Hysteria  may  simulate  each  of  these  pictures,  so  that  it  may  be  difficult 
to  distinguish  an  hysterical  from  an  organically  conditioned  (nervous) 
aphonia.  Organic  recurrens  paralyses  are  more  frequently  unilateral, 
hysterical  usually  bilateral;  but  one  must  remember  that  also  in  chronic 
catarrhs  slight  pareses  of  the  type  described  may  occur. 

Of  the  disturbances  in  speech,  the  most  important  group,  the  aphonic 
symptom  complex  belongs  to  a  special  chapter  (cf .  its  discussion  in  a  special 
part  of  the  volume) .  We  are  concerned  here  only  with  the  disturbances  in 
articulation  ("speech  utterance  disturbance"  " Sprachstoerung,"  Schuster) 
which  are  usually  designated  as  "anarthria"  or  "dysarthria."  It  is  a  ques- 
tion here  exclusively  of  innervation  disturbances  of  the  muscles  needed  in 
speech  (lips,  tongue,  palate,  also  larynx  and  respiration)  or  of  the  nerves 
subserving  them  (facialis,  hypoglossus,  vago-accessorius,  phrenicus)  and 
in  many  dysarthric  disturbances  one  can  recognize  at  once  from  the  act  of 
speech  which  group  of  muscles  is  attacked  when  the  patient  is  speaking. 
When  all  the  muscles  of  the  tongue,  palate  and  lips  are  affected,  there  results 
only  an  unintelligible  babbling,  total  anarthria,  as  we  usually  find  it  in 
the  final  stage  of  bulbar  paralysis.  But  this  disease  usually  shows  an  affec- 
tion of  the  separate  muscles  progressing  gradually  in  groups,  and  these  too 
are  not  easily  differentiated.  In  paralysis  of  the  tongue,  e  and  a,  also 
d,  1,  r,  s,  t,  are  spoken  indistinctly;  paralysis  of  the  lips  makes  the  utterance 
of,  00,  o,  b,  f,  p,  v,  difficult  or  impossible.  Paralysis  of  the  muscles  of  the 
soft  palate  makes  speech  nasal  (since  part  of  the  air  expired  passes  through 
the  nose  in  phonation) ,  and  separate  letters  (g,  k,  German  ch)  also  become 
indistinct. 


2o  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

Stuttering  is  dependent  upon  another  disturbance.  Here  it  is  a  matter, 
partly  of  a  tonic,  partly  of  a  clonic  spasm  in  the  muscles  used  in  speech,  and, 
moreover,  not  only  the  lips  and  tongue,  but  also  the  muscles  of  respiration 
are  affected  at  the  same  time.  Frequently  it  is  not  the  spasmodic  tension 
of  a  certain  muscular  group  that  takes  away  from  the  patient  the  possibility 
of  getting  beyond  the  initial  letter  (which  most  often  causes  the  trouble) 
but  the  synchronous  tension  of  various  muscles,  working  partly  as  antagonists. 
In  contrast  to  anarthria,  stuttering  must  be  looked  upon  as  a  functional 
neurosis,  and  is  scarcely  ever  dependent  upon  an  organic  disease. 

By  "scanning"  we  designate  a  speech  disturbance  in  which  the  single 
syllables  follow  one  another  slowly,  rhythmically,  at  clearly  defined  inter- 
vals. It  is  (with  rare  exceptions  in  hysteria)  an  organically  conditioned 
central  innervation  disturbance,  which  is  characteristic  of  multiple  sclerosis, 
and  finds  its  parallel  in  the  spastic  rigidity  of  the  muscles  of  the  extremities 
which  occurs  in  this  disease. 

The  stumbling  over  syllables  in  patients  suffering  from  progressive  paraly- 
sis, represents  a  mixture  of  anarthria  on  a  motor  and  on  a  psychic  basis. 
In  the  utterance  of  long  words — -favorite  words  for  the  test  are  "Massachu- 
setts artillery,"  "truly  rural" — the  first  syllables  are  spoken  correctly,  the 
following  ones  stumblingly,  or  are  mutilated  or  partly  omitted.  Obviously 
there  is  concerned  here  in  addition  to  the  mere  disturbance  in  articulation,  a 
forgetting  of  the  separate  syllables  during  speech,  or  failure  to  understand 
the  word.  Frequently  the  paralytic  does  not  notice  his  speech  disturbance, 
in  contrast  to  other  persons  suffering  from  dysarthria,  who  are  keenly  con- 
scious of  their  defect. 

Besides  the  disease  forms  already  mentioned,  we  find  anarthria  or 
dysarthria,  in  peripheral  nerve  affections  (hypoglossus),  occasionally  con- 
genital, and  commonly  in  all  sorts  of  diseases  of  the  cortex  of  the  cerebrum 
(tumors,  apoplexies);  in  these  latter,  frequently  with  a  genuine  aphasia, 
especially  in  recent  apoplexies,  which  must  not  be  overlooked  when  testing. 

Mutism,  the  total  inability  to  speak,  is  congenital,  except  for  its  appear- 
ance in  hysteria  and  will  be  discussed  in  that  chapter. 

5.  Disturbances  of  the  other  Cerebral  Nerves 

belong  here  only  in  the  minority  of  cases,  since  the  motor  nerves  (facialis, 
trigeminus  III,  accessorius,  hypoglossus)  will  be  mentioned  when  discussing 
motility,  the  sensory  trigeminus,  when  discussing  the  sensibility  test,  with 
the  symptoms  of  which  the  forms  of  disease,  in  the  main,  coincide.  But  we 
recommend  to  the  physician  who  is  making  a  systematic  examination  always 
to  institute  an  investigation  of  all  the  cerebral  nerves  preceding  that  of  the 
peripheral  nervous  system.  In  addition,  we  shall  mention  here  only  the 
disturbances  affecting  the  soft  palate,  and  the  complicated  mechanism 
representing  deglutition. 


DISTURBANCES  IN  SWALLOWING.    MOTILITY  21 

The  soft  palate  (mainly  innervated  by  the  vago-accessorius  group)  par- 
ticipates in  speaking,  swallowing  (and  in  opening  of  the  Eustachian  tube). 
We  find  corresponding  symptoms  of  disturbance:  1.  In  phonation  (one  has 
the  patient  pronounce  ah)  the  diseased  half  of  the  palate  is  not  raised,  but 
hangs  down  laxly,  the  soft  palate  and  the  uvula  are  in  a  position  slanting 
towards  the  sound  side  (the  uvula  even  in  normal  persons  is  not  always  quite 
straight).  2.  Upon  swallowing,  food  (especially  liquid  food)  passes  to  the 
nose  because  of  -  the  insufficient  closure  of  the  pharynx.  3.  Occasionally, 
slight  auditory  disturbance.  Paralyses  of  the  palate  are  found  most  fre- 
quently in  polyneuritis  (diphtheria),  then  in  central  affections  (bulbar 
paralysis) . 

In  deglutition  the  muscles  of  the  lips,  tongue,  palate,  pharynx  and 
oesophagus  are  involved.  Consequently,  different  disturbances  in  swallowing 
appear,  according  as  to  whether  only  one  and  which  one  of  these  groups  is 
affected.  In  weakness  of  the  lips,  liquid  flows  out  of  the  mouth,  in  weakness 
of  the  tongue,  it  cannot  be  transmitted  to  the  pharynx,  or  the  food  remains 
in  the  cavities  of  the  cheeks. 

Since  all  swallowing  of  liquids  is  effected  by  means  of  pressure  of  the 
tongue  against  the  hard  palate,  patients  with  paralysis  of  the  tongue  are 
often  unable  to  take  any  liquid  nourishment  at  all;  paralysis  of  the  palate 
causes,  as  has  been  said,  part  of  the  food  to  pass  into  the  nose.  Finally,  a 
paralysis  of  the  muscles  of  the  pharynx  and  oesophagus  causes  only  a  dis- 
turbance in  the  swallowing  of  solid  food,  which,  in  such  cases,  is  pushed 
down  only  with  great  trouble,  that  is,  sinks  down  of  itself.  Unilateral 
deglutition  paralysis  gives  rise  to  almost  the  same  disturbances  and  produces 
the  feeling  of  "  food  going  down  the  wrong  way,"  which  is  caused  by  entrance 
of  part  of  the  food  into  the  larynx  and  its  reflex  reaction. 

Disturbances  in  deglutition  are  found  most  frequently  in  toxic  and  infec- 
tious polyneuritis  and  in  central  nervous  diseases  (bulbar  paralysis,  etc.). 

Other  symptoms  of  the  glosso-pharyngeal-vagus  group  will  be  mentioned 
in  the  special  sections  devoted  to  neuritis  of  the  cerebral  nerves. 

We  may  remark  here  in  anticipation  that  electric  changes  in  the  cerebral 
nerves  may  be  established  with  certainty  only  in  the  facialis,  trigeminus  III, 
accessorius,  hypoglossus,  and  the  soft  palate. 

C.  Motility 

The  condition  and  activity  of  the  musculature  represents  the  first  of  the 
three  main  groups  in  the  examination  of  the  nervous  system  (especially  of 
the  peripheral). 

Inspection  of  the  musculature  can  distinguish,  above  all,  three  things — 
hypertrophy,  atrophy,  and,  to  a  certain  degree,  the  tonus  of  the  muscles.  To 
ascertain  the  presence  of  hypertrophy  or  atrophy,  especially  the  pathological 


22  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

degrees  of  both,  one  naturally  needs  an  exact  knowledge  of  the  normal 
forms  of  the  body,  that  is,  of  the  normal  (or  nearly  normal)  forms  of  the  mus- 
cles. Now  there  is  scarcely  any  other  tissue  of  the  body  which,  through 
exercise  or  lack  of  exercise  or  through  weakening  diseases,  etc.,  undergoes 
greater  changes  than  the  musculature.  The  great  muscles  of  the  pugilists 
may  bear  the  most  striking  external  similarity  to  the  pseudo-hypertrophic 
muscles  of  the  sufferer  from  dystrophy,  and  the  patient  with  immobilized 
joints  may,  in  respect  to  the  appearance  of  his  muscles  very  easily  be  con- 
fused at  first  sight  with  a  case  of  severe  polyneuritis.  A  less  practised  eye 
than  is  concerned  with  the  above  changes  is,  however,  somewhat  more 
useful,  if  it  be  a  question  of  recognition  of  differences  in  the  musculature 
between  the  right  and  the  left  side,  between  peripheral  and  central  parts. 
But  here,  too,  the  eye  alone  must  not  decide,  though  doubtless  it  best  recog- 
nizes slight  differences.  One  must  employ  an  exact  measurement  likewise, 
which,  in  so  far  as  musculature  alone  is  concerned,  usually  signifies  only 
measuring  the  circumferences  of  the  extremities.  Naturally,  for  measuring, 
exact  symmetrical  points  must  be  chosen  (measuring  the  distance  from 
fixed  points  upon  the  bones  in  centimeters),  and  it  must  be  considered 
furthermore  that  in  right  handed  persons  the  right  arm  and  the  right  leg 
have  a  circumference  of  about  one  to  two  cm.  more  than  the  corresponding 
extremities  upon  the  left  side.  The  trained  eye  can  naturally  detect  easily 
symmetrical  atrophies,  especially  those  in  the  extremities  (hands),  but  to 
establish  these  changes  definitely,  one  needs  also  an  exact  motility  and 
electricity  test.     For  tonus  of  the  muscles  cf.  below. 

Palpation  of  the  muscles  except  for  the  rare  determination  of  nodular 
thickenings  (tumors,  pseudo-hypertrophy)  or  a  general  softening  of  the 
muscular  substance,  affords  no  special  conclusions: 

All  the  more  important  is  the  motility  test.  In  order  to  control  this, 
one  naturally  needs  an  exact  knowledge  of  the  function  of  the  individual 
muscles,  which  shall  be  here  recapitulated  briefly,  in  so  far  as  it  is  concerned 
with  testing  the  active  movements.  (I  follow  in  the  main  StrilmpelVs 
division.) 

i.  Mimetic  musculature  (nervus  facialis). 

M.  frontalis. 

Function:  Wrinkles  the  forehead  into  transverse  folds. 

Test:  To  look  directly  upwards.     To  draw  up  the  eyebrows. 

Loss  in  paralysis:  After  the  order  to  draw  up  the  eyebrows,  absence  of 
wrinkling  on  the  diseased  side. 

M.  occipitalis:  Involved  in  the  wrinkling  of  the  forehead  and  the  scalp. 

Condition  in  loss  and  test  the  same  as  above. 

M.  corrugator  supercilii. 

Function:  Wrinkles  the  forehead  perpendicularly. 

Test:  Wrinkle  your  forehead! 


FUNCTIONS  OF  SINGLE  MUSCLES  23 

Loss  in  paralysis:  In  unilateral  paralysis,  an  often  indistinct  absence 
of  wrinkles  during  the  test. 

M.  orbicularis  oculi. 

Function:  Eye  closure. 

Test:  Close  your  eyes! 

Loss  in  paralysis:  The  palpebral  fissure  can  not  be  completely  closed. 
Lagophthalmos. 

M .  compressor  nasi,  levator  alee  nasi,  zy gomaticus ,  risorius,  levator  labii 
sup. 

Function:  Elevating  the  alas  nasi  and  the  corner  of  the  mouth. 

Test:  Turn  up  your  nose! 

Functional  loss  in  paralysis:  Corresponding;  above  all,  loss  of  the  naso- 
labial fold. 

M.  orbicularis  oris. 

Function:  Pursing  the  lips,  whistling. 

Test:  Corresponding  command. 

Functional  loss  in  paralysis:  Disability  or  inability  to  move  the  lips. 

Atrophy  of  the  mimetic  muscles  leads  to  sinking  in  of  the  parts  involved. 

2.  Chewing  (masticatory)  musculature. 

Mm.  masseter  and  temporalis  (N.  Tringeminus  III) . 
Function:  Movements  of  chewing. 
Test:  Close  your  teeth  tightly ! 

Functional  loss  in  paralysis:  Noticeable  gap  in  the  place  of  both  muscles 
on  the  diseased  side.     Especially  distinct  in  simultaneous  atrophy. 

3.  Movement  of  the  soft  palate;  deglutition:  cf.  above,  under  "other 
cerebral  nerves." 

4.  Muscles  of  the  tongue  (N.  hypoglossus) . 
Function:  All  independent  movements  of  the  tongue. 

Test:  Stick  out  your  tongue,  move  it  to  the  right,  to  the  left,  roll  it  up- 
wards, downwards! 

Functional  loss  in  paralysis:  In  unilateral  paralysis,  the  tip  of  the  out- 
stretched tongue  deviates  towards  the  paralyzed  side  (as  a  result  of  the 
peculiar  radial  distribution  of  the  m.  genioglossus) .  The  movements  of  the 
tongue  are  all  more  or  less  hindered,  but  an  isolated  test  of  the  other  muscles 
of  the  tongue  (m.  lingualis,  m.  transversus  linguae)  is  of  no  great  importance. 
In  bilateral  paralysis  (bulbar  paralysis,  etc.),  the  tongue  lies  motionless  upon 
the  floor  of  the  buccal  cavity.  Atrophy  of  the  musculature  of  the  tongue 
leads  to  the  quite  characteristic  picture  of  the  furrowed  tongue,  which  shows 
uni-  or  bilateral  transverse  folds,  irregular  elevations  and  depressions  and 
feels  abnormally  soft. 

5.  M.  sterno-cleido-mastoideus  (N.  accessorius) . 

Function:  Rotates  and  partially  moves  the  head  forward. 

Test:  Press  the  chin  hard  upon  the  fist  of  the  examiner. 


24  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

Functional  loss  in  paralysis:  The  head  is  incompletely  rotated,  and  bent 
crookedly  forwards  (the  chin  turns  towards  the  paralyzed  side) .  In  the  test 
described  the  characteristic  cord  of  the  muscle  appears  only  on  the  sound 
side. 

6.  Muscles  of  the  Back. 

Mm.  splenii,  biventer,  recti  capitis  postici  (cervical  nerves  1-4) . 
Function:  Backward  movement  of  the  head  and  the  cervical  vertebras. 
Test:  Lay  the  head  backwards  upon  the  supporting  hand  of  the  examiner. 
Functional  loss  in  paralysis:  Oblique  and  incomplete  performance  of 
this  movement. 

7.  Mm.  sacrolumbalis,  longissimus  dorsi,  spinalis  dorsi  (spinal 
nerves) . 

Function:  Erects  the  spinal  column. 

Test:  Rise  from  a  stooping  position  against  the  resisting  hand  of  the 
examiner,  without  help  from  the  hands.     (But  cf.  under  34.) 

Functional  loss  in  paralysis:  Hindrance  or  inability  to  rise  in  the  test 
described.  In  unilateral  paralysis  dorsolumbar  scoliosis  (often  with  lordo- 
sis) with  convexity  towards  the  paralyzed  side.  In  bilateral  paralysis: 
severe  lumbar  lordosis  in  walking  (gait  of  dystrophics);  in  sitting  often 
(not  always)  kyphosis. 

8.  Abdominal  muscles  (recti,  obliqui,  transversus  abd. ;  dorsal 
nerves  from  the  eighth  on) . 

Function:  Abdominal  pressure;  raising  the  body  from  a  supine  position. 
Flexing  the  spinal  column  forwards. 

Test:  Exert  pressure  as  if  evacuating  the  bowels.  Rise  from  a  supine 
position  without  help  from  the  hands. 

Functional  loss  in  paralysis:  Impossibility  of  rising  as  described.  Diffi- 
culty in  evacuation  of  the  feces  and  urine.  Lumbar  lordosis,,  pot-belly. 
In  unilateral  paralysis,  occasionally  the  linea  alba  and  navel  are  drawn 
towards  the  sound  side. 

9.  M.  quadratus  lumborum  (plexus  cruralis). 
Function:  Lateral  movement  of  the  spinal  column. 
Test:  Corresponding  to  the  function. 

Functional  loss  in  paralysis:  Corresponding,  generally  unimportant. 

10.  Diaphragm  (N.  phrenicus  from  cervic.  4). 
Function:  Lengthening  of  the  thoracic  cavity  in  inspiration. 
Test:  Breathe  deeply. 

Functional  loss  in  paralysis:  Absence  of  the  epigastric  protuberance, 
retraction  of  the  intestines  and  the  diaphragm  phenomenon  (visible  descend- 
ing shadow  upon  lateral  illumination)  in  inspiration;  dyspnoea. 

11.  Muscles  of  the  shoulder  girdle  and  arm. 
M.  cucullaris  or  trapezius  (N.  accessorius) . 

Function:  Elevates  the  shoulder  blade  and  brings  it  nearer  to  the  middle'line. 


FUNCTIONS  OF  SINGLE  MUSCLES  25 

Test:  Raise  your  shoulder  blades  ("  shrug  your  shoulders  ") ! 

Functional  loss  in  paralysis:  Since  frequently  only  one  of  the  three  por- 
tions of  the  muscle  is  paralyzed,  the  picture  may  vary.  The  characteristic 
of  cucullaris  paralysis,  restriction  in  raising  the  shoulders,  belongs  actually 
to  the  middle  portions  alone,  whereas  the  upper  (clavicular)  only  when  the 
shoulder  is  fixated,  draws  the  head  somewhat  towards  the  back,  and  the 
lowest  causes  the  shoulder  to  move  towards  the  spinal  column.  The  typical 
picture  of  total  paralysis  of  the  muscles  is :  sinking  of  the  acromion,  lowering 
and  forward  position  of  the  shoulder  (and  the  arm),  more  or  less  pronounced 
horizontal  position  of  the  collar  bone,  and  a  moving  away  of  the  scapula 
from  the  spinal  column.  The  atrophy  of  the  muscle,  which  is  usually  present, 
leads  to  a  flattening  of  the  line  between  the  shoulder  and  the  neck  and  to  a 
clearer  prominence  of  the  contours  of  the  scapula  (above  all  of  the  spine  of 
that  bone).  This  position  of  the  shoulder  blade  is  designated  as  "angel- 
winged." 

12.  M.  levator  anguli  scapulae (  1  to  3  cervicalis). 
Function:  Elevates  the  inner  superior  angle  of  the  scapula. 

Test:  Raise  your  shoulders.  (Usually,  if  the  cucullaris  is  retained, 
not  easily  recognizable) ;  when  the  cucullaris  is  paralyzed,  partly  replaces 
it  functionally. 

Functional  loss  in  paralysis:  Slight  (in  so  far  as  it  is  isolated) .  In  com- 
bination with  cucullaris  paralysis;  inability  to  raise  the  scapula. 

13.  Mm.  rhomboidei  (4  to  5  cervicalis) . 

Function:  To  bring  the  scapula  nearer  to  the  spinal  column  (especially 
the  inferior  angle). 

Test:  Draw  your  shoulders  together  in  the  back! 

Functional  loss  in  paralysis:  The  inner  border  of  the  shoulder  blade 
stands  out  from  the  thorax  (visible  almost  only  when  simultaneous  cucullaris 
paralysis  exists). 

14.  M.  serratus  anticus  major  (X.  thoracicus  longus,  from  cervical  5). 
Function:  Rotates  the  scapula  about  the  sagittal  axis,  and  fixates  it 

when  the  upper  arm  is  raised  vertically;  serves  for  fixation  of  the  scapula 
to  the  thorax,  even  in  a  state  of  rest. 

Test:  Raise  your  arm  above  the  horizontal  position.  Press  your  arm 
forwards  against  some  resistance  (pushing). 

Functional  loss  in  paralysis:  In  rest,  frequently,  a  deviated  position  of  the 
inner  margin  of  the  scapula,  which,  below,  draws  nearer  the  spinal  column, 
at  the  same  time  rising  a  little  from  the  thorax.  The  arm  can  no  longer  be 
raised  above  the  horizontal  line  because  of  the  insufficient  fixation  of  the 
scapula.  In  pushing  the  arm  forwards,  the  inner  margin  of  the  scapula 
moves  forward  like  a  wing  from  the  thorax:  "winged  shoulder."  The 
atrophy  that  is  usually  present  at  the  same  time  allows  the  winged  position 


26  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

to  become  more  clearly  visible,  and  brings  about  a  recognition  of  the  usually 
easily  seen  serratus  digitations  in  the  lateral  wall  of  the  thorax. 

15.  M.  deltoideus  (N.  axillaris). 

Function:  Elevates  the  arm  to  somewhat  above  the  horizontal  line. 

Test:  Raise  your  arm  to  a  horizontal  position  (eventually  against 
resistance). 

Functional  loss  in  paralysis:  The  arm  can  not  be  raised.  The  usually 
simultaneous  atrophy  is  characterized  by  loss  of  the  shoulder  curve  and  by 
prominence  of  acromion  and  caput  humeri. 

16.  M.  pectoralis  major  and  minor  (N.  thoracici  ant.  from  cervical  5 
and  6). 

Function:  Draws  the  arm  up  to  the  thorax. 

Test:  Press  your  outstretched  arm  against  some  resistance  (hands  of 
the  examiner). 

Functional  loss  in  paralysis:  Usually  only  weakness  in  the  before  men- 
tioned adduction  movement,  since  deltoideus  and  teres  major  afford  partial 
compensation.    In  severe  atrophy,  the  upper  ribs  may  stand  out  prominently. 

17.  M.  latissimus  dorsi  (N.  subscapularis  from  cervicalis  5  and  6). 
Function:  Draws  the  upper  arm  backward  and  downward. 

Test:  With  horizontally  raised  upper  arm,  press  downward  and  back- 
ward upon  the  supporting  hand  of  the  examiner. 

Functional  loss  in  paralysis:  Weakness  in  the  test  described. 

18.  Mm.  supraspinatus,  infraspinatus,  teres  minor  (N.  supra- 
scapular). 

Function:  Rotates  the  arm  outward. 

Test:  Rotate  the  outstretched  arm  outward  (against  resistance). 

Functional  loss  in  paralysis:  Weakness  in  the  movement  described. 
Sometimes  disturbance  in  writing.  The  frequently  simultaneous  atrophy 
may  be  recognized  by  the  flattening  of  the  posterior  arch  of  the  shoulder 
blade. 

19.  M.  subscapularis,  teres  major  (N.  subscapularis). 
Function:  Rotates  the  arm  inwards. 

Test:  Rotate  the  extended  arm  inwards  (against  resistance). 
Functional  loss  in  paralysis:  Weakness  of  the  movement  described. 

20.  Mm.  biceps,  brachialis  internus  (N.  musculocutaneous). 
Function:  Flexes  the  forearm  towards  the  upper  arm  without  any  rota- 
tion of  the  lower  arm  (slight  supination). 

Test:  Flex  your  forearm  when  in  a  supine  position  (against  resistance). 

Functional  loss  in  paralysis:  Impossibility  of  flexing  in  this  position; 
there  appears  at  once  pronation  of  the  forearm.  Atrophy  of  these  muscles 
is  easily  recognized  by  the  loss  of  the  biceps  curvature;  the  circumference 
of  the  upper  arm  is  decreased  perceptibly. 

21.  M.  supinator  longus  (N.  fadialis). 


FUNCTIONS  OF  SINGLE  MUSCLES  27 

Function:  Flexes  the  forearm  towards  the  upper  arm  in  half  pronated 
position  (the  middle  position  between  pronation  and  supination). 

Test:  Flex  your  forearm  against  resistance  with  half  pronated  hand 
(palms  towards  the  inside). 

Functional  loss  in  paralysis:  In  attempts  at  movement  the  hand  is  at 
once  supinated.  Atrophy  can  be  easily  recognized  by  a  conspicuous  gap 
in  the  musculature  on  the  radial  side  of  the  bend  of  the  elbow. 

22.  M.  triceps  (N.  radialis). 
Function:  Extends  the  forearm. 

Test:  Extend  your  flexed  arm  against  resistance. 

Functional  loss  in  paralysis:  Impossibility  of  performing  this  movement 
against  resistance. 

23.  Mm.  extensores  carpi  (rad.  and  uln.,  N.  radialis). 

Function:  Extends  (that  is,  overextends) ,  the  hand  towards  the  forearm. 
Test:  Extend  your  hand  against  resistance  (at  the  metacarpus). 
Functional  loss  in  paralysis:  Impossibility  of  performing  the  movement 
described;  the  hand  hangs  down  limply  from  the  extended,  pronated,  forearm. 

24.  Mm.  ext.  digit,  comm.,  indicator,  ext.  digiti  v  (N.  radialis). 

Function:  Extends  the  basal  phalanges  of  the  second  to  fifth  fingers. 

Test:  Extend  your  fingers  against  resistance  (at  the  basal  phalanges). 

Functional  loss  in  paralysis:  Impossibility  of  performing  the  move- 
ment described.  The  atrophy  of  all  the  muscles  mentioned  under  23 
and  24  causes  usually  a  moderate  degree  of  wasting  in  the  dorsal  aspect  of 
the  forearm. 

25.  Mm.  interossei  ext.,  and  int.,  Mm.  lumbricales  (N.  ulnaris,  also 
medianus) . 

Function:  These  groups  of  muscles  abduct  and  adduct  the  fingers  (only 
the  interossei)  at  the  same  time,  flex  the  basal  phalanges,  and  extend  the 
end  phalanges  of  the  fingers. 

Test:  Spread  and  press  together  against  resistance  the  fingers  that  have 
been  extended  (it  is  best  to  do  this  against  the  fingers  of  the  examiner  which 
are  inserted  as  in  "folding  the  hands").  Flex  the  fingers  against  resistance 
(at  the  basal  phalanges)  and  extend  them  against  resistance  (at  the  end 
phalanges). 

Functional  loss  in  paralysis:  Impossibility  of  performing  the  movements 
described;  above  all,  inability  to  hold  on  to  thin  objects  (needle,  coins)  in 
the  fingers.  In  a  condition  of  rest,  the  4th  and  5th  fingers  deviate  towards 
the  ulnar  side.  There  is  no  strength  in  pressure  of  the  hand.  Atrophy  is 
made  very  typical  by  the  sinking  in  of  the  spatia  interossea  and  by  the 
hyperextension  of  the  basal  phalanges  resulting  from  the  preponderance 
of  the  antagonists  (the  long  extensors  and  flexors)  with  simultaneous  flexion 
of  the  end  phalanges.  Wasting  of  the  palm.  The  type  of  hand  known  as 
"claw  hand"  (paralysis  of  the  ulnaris). 


28  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

26.  M.  palmaris,  Mm.  flexores  carpi  (rad.  and  uln.,  N.  medianus  and 
n.  ulnaris). 

Function:  Flexes  the  hand  towards  the  forearm. 
Test:  Flex  your  hand  against  resistance  (at  the  metacarpus). 
Functional  loss  in  paralysis:  Usually  slight;  weakness  of  the  movement 
mentioned. 

27.  Mm.  flexores  digit,  long.  subl.  and  profund  (N.  medianus). 
Function:  Flex  the  middle  (sublimis)  and  end  phalanges  (profundus). 
Test:  Pressure  of  the  hand  with  co-operation  of  the  lumbricales  and 

interossei.  Eventually,  isolated  test  of  the  profundus:  pressing  the  tips 
of  the  fingers  against  tips  of  the  flexed  fingers  of  the  inverted  hand  of  the 
examiner,  with  attempts  to  flex  the  fingers  (interlocking). 

Functional  loss  in  paralysis:  Weakness  of  hand  pressure.  One  must 
observe  that  simultaneous  weakness  of  the  extensores  carpi  also  simulates 
weak  hand  pressure,  since  when  the  hand  hangs  down,  the  flexors  mentioned 
are  unable  to  functionate.  Therefore  with  simultaneous  radialis  paralysis, 
the  test  can  be  made  only  with  passively  fixed  wrist.  Atrophy  of  26  and  27 
combined,  cause  wasting  of  the  forearm  (usually  of  moderate  degree). 

28.  M.  flexor  brevis  and  abductor  digiti  minimi  (N.  ulnaris). 
Function:  Flexes  the  basal  phalanx  of  the  little  finger  and  abducts  the 

little  finger. 

Test:  Flex  your  little  finger  against  resistance  (hooked  in  the  finger  of 
the  examiner). 

Functional  loss  in  paralysis:  Weakness  of  the  movement  mentioned. 
Atrophy  of  these  muscles  mainly  causes  the  characteristic  flattening  of  the 
ball  of  the  little  finger  in  paralysis  of  the  ulnaris. 

29.  Mm.  extensor  pollicis  brevis  and  longus,  M.  abductor  pollicis 
(N.  radialis). 

Function:  Extension,  that  is,  abduction  of  the  thumb  and  the  metacarpus. 
Test:  Extend  and  abduct  the  flexed  thumb  against  resistance. 
Functional  loss  in  paralysis:  Weakness  of  the  movement  described.     The 
thumb  falls  into  the  palm  of  the  hand. 

30.  M.  adductor  pollicis  (N.  ulnaris). 

Function:  Adducts  the  thumb  (metacarpus)  towards  the  forefinger. 
Test:  Press  your  thumb  against  resistance  towards  the  fixed  forefinger. 
Functional  loss  in  paralysis:  Weakness  of  the  movement  mentioned. 

31.  M.  opponens,  abductor  brevis  and  flexor  brevis  pollicis  (N. 
medianus). 

Function:  Flexion  and  opposition  of  the  metacarpus  1  and  the  thumb 
(1  phalanx). 

Test:  Press  your  hand. 

Functional  loss  in  paralysis:  Weakness  in  hand  pressure.  Often  dis- 
turbances in  writing.     The  atrophy,  which  is  usually  present  at  the  same  time, 


FUNCTIONS  OF  SINGLE  MUSCLES  29 

flattens  the  ball  of  the  thumb  in  characteristic  fashion,  and  gradually -brings 
the  thumb  into  the  direction  of  the  other  fingers,  in  the  plane  of  which  it 
finally  lies:  "ape  hand"  of  the  medianus  paralysis. 

32.  Muscles  of  the  leg. 

M.  ileopsoas   (N.  cruralis),  M.  tensor  fascia  lata  (1   and  2  lumbalis). 
Function:  Elevates  the  leg  at  the  hip  joint. 

Test:  Raise  your  extended  leg  from  a  supine  position  (against  resistance). 

Functional  loss  in  paralysis:  Disturbance  in  walking;  walking  is  usually 

quite  impossible,  as  is  also  raising  the  extended  leg  from  a  supine  position. 

33.  M.  sartorius  (N.  cruralis). 

Function:  Test  like  that  under  ^2.  Not  infrequently,  in  paralysis  of 
the  flexors  of  the  thigh  and  of  the  extensors  of  the  leg  proper  the  sartorius 
alone  is  spared. 

34.  Mm.  glutaei  (sacral  nerves). 

Function:  Extension  and  slight  abduction  of  the  leg  at  the  hip  joint. 

Test:  In  a  supine  position,  press  down  against  resistance  the  raised 
thigh.  When  standing,  rise  from  a  stooping  position  against  resistance. 
Press  the  thigh  outwards  against  resistance. 

Functional  loss  in  paralysis:  Difficulty  in  rising  from  a  sitting  posture, 
in  mounting  stairs,  in  rising  from  a  bent  position  (in  common  with  7),  wad- 
dling gait. 

35.  Mm.  adductor  brevis,  longus,  magnus,  pectineus,  gracilis  (N. 
obturatorius) . 

Function:  Adduction  of  the  thigh  at  the  hip  joint. 
Test:  Press  the  thighs  together  against  resistance. 

Functional  loss  in  paralysis:  Loss  of  the  movement  mentioned.  Inse- 
cure seat  when  riding  horseback. 

36.  M.  extensor  cruris  quadriceps  (N.  cruralis). 
Function:  Extends  the  leg  proper  at  the  knee  joint. 

Test:  In  a  supine  position,  extend  the  leg  against  resistance,  while  the 
arm  of  the  examiner  placed  below  the  knee  fixates  the  thigh  in  a  slightly 
flexed  position. 

Functional  loss  in  paralysis:  Impossibility  of  extending  the  leg  proper  in 
the  manner  described.  Severe  disturbance  in  walking,  though  walking  is 
possible  with  the  leg  in  extension,  as  long  as  the  patient  seemingly  supports 
himself  upon  the  surface  of  the  knee  joint  and  does  not  innervate  the  flexors 
of  the  leg  proper.     Mounting  stairs  is,  as  a  rule,  impossible. 

37.  M.  biceps,  semitendinosus,  semimembranosus  (N.  ischiadicus) . 
Function:  Flexion  of  the  leg  proper  and  extension  of  the  hip  joint. 
Test:  In  a  supine  position,  draw  up  the  leg  (against  resistance)  towards 

the  thigh. 

Functional  loss  in  paralysis:  Inability  to  perform  the  movement  men- 
tioned, moderate  disturbance  in  gait,  inability  to  jump  and  run. 


30  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

38.  M.  tibialis  anticus  (N.  peroneus). 

Function:  Elevates  the  inner  margin  of  the  foot  and  extends  the  foot 
(subjects  it  to  dorsal  flexion). 

Test:  Scarcely  possible  as  an  isolated  movement. 

Functional  loss  in  paralysis:  Inconsiderable  in  isolated  paralysis.  The 
atrophy  effects  a  characteristic  sinking  in  of  the  muscular  layer  directly 
next  to  the  margin  of  the  tibia.  Frequently  the  tendon  of  the  overexerted 
ext.  hallucis  stands  out  prominently;  the  large  toe  is  flexed  dorsally. 

39.  M.  peroneus  longus  and  brevis  (N.  peroneus). 

Function:  Elevates  the  outer  margin  of  the  foot  and  subjects  the  foot  to 
slight  flexion  (plantar  flexion). 

Test:  As  an  isolated  movement  only  possible  electrically. 

Functional  loss  in  paralysis:  In  attempting  extension,  adduction  of  the 
foot  appears;  the  great  toe  drags  slightly  upon  the  ground  in  walking;  flat- 
foot  develops. 

40.  Mm.  extensor  digit,  comm.  longus,  ext.  hallucis  longus  (N. 
peroneus). 

Function:  Elevates  the  toes  and  thereby  the  tip  of  the  foot. 

Test:  Draw  up  the  toes  against  resistance. 

Functional  loss  in  paralysis:  The  toes  hang  down  limply.  The  par- 
ticipation of  these  muscles  effects,  mainly,  however,  in  combination  with  38 
and  39,  the  picture  of  the  peroneus  paralysis:  the  tip  of  the  foot  hangs  limply 
down,  usually  its  inner  margin  is  the  lowest;  as  a  result,  it  always  drags 
upon  the  ground  in  walking.  In  order  to  avoid  this  the  patient  raises  his 
thigh  excessively  when  walking,  the  characteristic  "steppage  gait"  results. 
Through  secondary  contracture  of  the  antagonists  (calf)  there  frequently 
develops  pes  equinus,  or  pes  equino-varus.  Atrophy  of  the  anterior  aspect 
of  the  leg  proper. 

41.  M.  triceps  surae  (M.  gastrocnemius,  plantaris,  soleus — N.  tibialis). 
Function:  Plantar  flexion  of  the  foot. 

Test:  Press  the  foot  downwards  against  the  hand  of  the  examiner; 
stand  upon  the  tips  of  the  toes. 

Functional  loss  in  paralysis:  Loss  of  flexion  of  the  foot.  Inability  to 
stand  upon  the  tips  of  the  toes  or  to  dance.  There  frequently  develops  a  pes 
calcaneus.  Atrophy  of  this  muscular  group  causes  the  most  severe  atrophy 
possible  in  the  leg  proper. 

42.  M.  flexor  dig.  comm.  longus,  brevis,  flexor  hallucis  longus 
and  brevis  (N.  tibialis). 

Function:  Plantar  flexion  of  the  toes. 

Test:  Flex  the  toes  downwards  against  resistance. 

Functional  loss  in  paralysis:  Inconsiderable. 

43.  Mm.  interossei  (N.  tibialis). 
Function:  Flexion  of  the  basal  phalanges. 


TYPES  OF  PARALYSIS  31 

Test:  Scarcely  possible  in  isolation. 

Functional  loss  in  paralysis:  Inconsiderable.  But  through  the  atrophy 
of  these  muscles  and  secondary  contractures  of  the  antagonists,  atrophy 
of  the  planta  pedis  and  a  "claw-foot"  may  develop. 

Functional  testing  conducted  in  this  way  will  always  give  a  complete 
picture  of  the  power  of  the  individual  muscles.  For  separate  groups  of 
muscles,  functional  power  may  be  measured  directly  by  means  of  the  so- 
called  dynamometer  (Charriere,  Duchenne,  Sternberg),  which,  however,  has 
been  extensively  used  only  for  pressure  of  the  hands.  Here  it  gives  good 
comparative  values  in  respect  to  the  strength  of  both  hands  and  affords  a 
comparison  of  the  strength  in  various  stages  of  the  same  disease. 

The  degree  of  the  paralyses  is  important  for  their  recognition.  While 
total  paralyses  even  of  individual  muscles  alone  will  scarcely  escape  the  notice 
of  the  examiner,  mere  conditions  of  weakness,  pareses,  are  often  very  difficult 
to  recognize;  they  are  most  easily  recognized  in  unilateral  paralyses  through 
comparison  with  the  same  muscle  on  the  other  side.  Naturally  disturbances 
of  the  sensorium  cause  special  difficulties  in  the  recognition  of  palsies;  here 
sometimes  the  tonus  (cf.  below)  is  of  decisive  importance;  in  recent  apoplex- 
ies, the  raised  arm,  even  in  stuporous  patients,  falls  down  far  more  laxly  on 
the  paralyzed  side,  as  if  dead,  in  contrast  to  that  on  the  sound  side.  In  small 
children,  the  determination  even  of  complete  paralyses  is  often  difficult. 
Where  it  is  not  a  matter  of  the  more  easily  recognized  disturbances  in  walking, 
the  reaction  of  the  child  to  pain  stimulations  often  leads  to  the  goal;  one 
pinches  or  pricks  the  skin  approximately  in  the  region  of  the  antagonists  of 
those  groups  of  muscles  that  one  wishes  to  test;  the  degree  of  reaction  to 
escape  the  irritation  will  usually  determine  whether  or  not  weakness  is 
present. 

As  to  the  type  of  paralysis,  naturally  nothing  has  been  ascertained  by  the 
mere  determination  of  its  presence.  We  must  emphasize  here,  that  almost 
every  muscular  paralysis  may  be  of  organic  or  "functional"  (psychogenous, 
usually  hysterical)  origin;  but  even  among  the  organic  paralyses,  there  is 
scarcely  any  point  in  the  entire  nerve-muscular  apparatus  which  may  not 
give  rise  to  muscular  paralyses:  disease  of  the  muscle  itself  (myositis, 
possibly  dystrophy  and  myotonia) ,  of  the  peripheral  nerve  (neuritides) ,  of  the 
spinal  cord  (diseases  of  the  columns  of  the  spinal  cord,  poliomyelitis,  etc.), 
of  the  medulla,  oblongata  and  brain  (bulbar  paralyses,  apoplexies,  etc.). 
The  paralysis  may  furthermore,  as  we  shall  see  below,  he  flaccid  or  spastic 
(with  diminution  or  increase  of  the  tonus),  may  be  accompanied  by  atrophy 
or  hypertrophy,  etc.  As  to  what  form  is  concerned  in  the  individual 
case,  frequently  electrical  testing,  sometimes  only  observation  of  the 
entire  disease  picture  may  decide. 

In  our  discussion  of  the  individual  muscles,  we  have  always  noted  when 
the  atrophy  of  a  muscle  produces  characteristic  changes  inform.     Now  this 


32  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

happens  more  frequently  in  groups  of  muscles  than  in  individual  muscles; 
their  decrease  in  volume  produces  characteristic  pictures,  which,  for  the  experi- 
enced physician,  make  possible  diagnosis  at  first  sight.  The  pictures  are 
often  typical  of  certain  well-determined  diseases.  We  mentioned  the  "claw- 
hand"  of  ulnar  paralysis,  the  "ape-hand"  of  median  paralysis  (of  high  degree), 
dorsal  flexion  of  the  large  toe  in  paralysis  of  the  tibialis  anticus,  etc.  These 
pictures  are  found  most  frequently,  especially  in  isolated  forms,  in  peripheral 
neuritis,  somewhat  less  frequently  (and  scarcely  ever  alone)  in  poliomyelitis, 
syringomyelia,  bulbar  paralysis.  For  progressive  neurotic  muscular  atrophy, 
the  peripheral  beginning  of  the  atrophy  is  characteristic;  the  extremities 
acquire  that  peculiar  attenuation  towards  the  periphery,  which  is  known  as 
"bird-legs."  On  the  other  hand,  progressive  spinal  muscular  atrophy  also 
attacks  the  periphery  first,  but  the  atrophy  here  is  of  much  higher  degree. 
In  syringomyelia  we  see  that,  as  a  rule,  only  the  upper  extremities  show  mus- 
cular atrophy;  for  poliomyelitis  the  apparently  total  participation  of  all  pos- 
sible muscular  groups  here  and  there,  all  over  the  entire  body  is  characteristic. 
The  most  various  and  most  easily  remembered  pictures  are  presented  prob- 
ably by  dystrophy;  the  thin  upper  arms  with  the  strong  lower  arm,  the  winged 
shoulders,  lumbar  lordosis,  if  the  face  is  affected  the  tapir-lip,  lagophthalmos. 
The  number  of  such  characteristic  pictures  may  be  easily  increased,  especially 
if  one  counts  in  all  the  changes  in  form  dependent  upon  contractures  (cf. 
below)  with  or  without  synchronous  atrophies.  For  the  sake  of  simplicity, 
we  may  here  mention  at  once  some  other  pictures  of  disturbances  in  gait, 
not  directly  connected  with  muscular  paralysis  and  atrophy.  We  have  just 
mentioned  the  "steppage"  gait,  the  waddling  gait,  posture  and  gait  in 
dystrophy.  These  kinds  of  gait  belong  to  the  simple  paretic  variety.  All 
gait  disturbances  accompanied  by  lesion  of  the  pyramidal  tracts  are  to  be 
designated  as  spastic,  or  spastic  paretic.  Common  to  them  are  spasms  or 
tensions  (cf.  under  hypertonia) ;  in  walking  the  legs  are  pushed  forwards  as  if 
they  were  made  of  wood,  flexed  but  slightly  at  the  hip  and  not  at  all  at  the 
knee,  the  tips  of  the  feet  graze  the  ground,  the  soles  of  the  shoes  are  always 
worn  through  at  a  definite  place.  Such  is  the  gait  in  spastic  spinal  paralysis, 
lateral  sclerosis,  myelitis,  mostly  too  in  syringomyelia  and  multiple  sclerosis. 
The  ataxic,  "swinging"  or  long-striding  gait  of  the  tabetics,  the  "tottering" . 
gait  of  cerebellar  ataxia,  are  mentioned  below  under  ataxia.  Persons  suffer- 
ing from  paralysis  agitans  walk  stiffly,  mostly  with  bent  back  and  bowed 
head,  taking  small  steps,  also  "stick"  to  the  ground,  and  still  the  gait  is  not 
really  spastic;  nay,  rather  upon  occasion  very  quick  tripping  steps  are  made 
forwards  and  backwards,  irresistibly,  especially  when  with  a  light  push,  one 
sends  the  patient  forward  or  backward  or  tells  him  to  run.  Then  he  cannot 
arrest  his  movements  (symptom  of  pro-  and  retropulsion),  which  never  occurs 
in  spastics.  Total  inability  to  walk,  known  as  "abasia"  ("astasia"),  which  is 
not  rare  in  hysteria,  will  be  mentioned  in  the  special  part  of  the  book. 


DISORDERS  OF  WRITING.     MUSCULAR  TONUS  33 

The  manifold  picture  of  writing  disturbances  of  a  motor  nature  furnish  a 
parallel  to  a  certain  extent  to  the  disturbances  in  gait.  Mere  paretic  disturb- 
ances, such  as  appear  in  isolated  median  or  ulnar  paralysis  are  but  little 
characteristic;  the  patients  write  slowly,  with  trouble,  but  the  writing  is  not 
characteristically  changed,  and  occasionally  retains  wholly  its  previous 
character.  Clumsiness  naturally  increases  in  complicated  pareses  (spinal 
muscular  atrophy,  multiple  neuritis) .  There  may  appear  total  motor  inabil- 
ity to  write  (motor  agraphia).  In  spastic  conditions  the  writing  as  a  rule 
becomes  smaller,  labored,  the  separate  letters  run  into  one  another,  and  the 
words  often  are  illegible.  Ataxic  script  (tabes  superior)  furnishes  the  type  of 
deviating  movements  on  paper;  the  letters  are  of  varying  length,  single  strokes 
pass  across  the  letters  already  written,  the  lines  are  crooked  and  vary  in 
level.  Very  similar  is  the  script  of  most  sufferers  from  writer's  cramp. 
In  multiple  sclerosis  the  letters  do  not  run  into  one  another,  but  the  script  is 
shaky,  and  there  are  very  regularly  found  deviations  corresponding  to  the 
tremor  in  the  stroke,  the  general  direction  of  which  is  correctly  maintained. 
Of  the  genuine  forms  of  tremor,  paralysis  agitans  has  the  most  typical  change 
in  script,  the  so-called  flower  script :  the  easily  legible  letters  in  all  their  parts 
show,  instead  of  straight  lines,  small  zig-zag  lines,  consisting  of  deviations  of 
the  length  of  a  millimeter.  For  the  writing  of  sufferers  from  progressive 
paralysis,  etc.,  cf.  the  special  chapters  concerned. 

Pathological  hypertrophies  are  much  rarer;  only  acromegaly  and  pseudo- 
hypertrophy in  dystrophy  are  really  concerned  here.  Both  likewise  give 
typical  pictures. 

A  symptom  of  great  importance  or  rather  the  nucleus  of  several  large 
groups  of  symptoms  is  muscular  tonus.  By  this  one  designates  the  con- 
dition of  a  certain  permanent  irritability,  so  to  speak,  in  the  sound 
muscle  (also  in  condition  of  rest),  which  we  may  interpret  as  a  reflex  pro- 
cess localized  in  the  ganglion  cells  of  the  anterior  horn  of  the  spinal  cord. 
In  this  process,  the  anterior  horns  represent  to  a  certain  extent  an  electrical 
power  station  (Schuster),  which  is  influenced  by  the  higher  centers  of  the 
cerebrum  as  well  as  by  the  periphery.  Peripheral  sensory  stimulations  may 
increase  temporarily  the  tonus  of  the  muscles  (precisely  by  way  of  this  power 
station) ,  as  well  as  may  the  impulse  of  the  will  emanating  from  the  cerebrum, 
evoking  the  voluntary  movement;  on  the  other  hand,  there  doubtless  exist  also 
between  the  cerebrum  and  anterior  horns  inhibiting  influences,  since  in 
interruption  of  this  conductivity  the  tonus  may  increase. 

As  to  the  tonus  of  the  musculature,  we  can  get  some  data  from  simply 
testing  the  musculature,  partly  also  from  mere  observation  of  the  patient. 
If  his  limbs  lie  flaccid,  touching  throughout  their  entire  extent  whatever  they 
are  resting  upon,  possibly  exhibiting  also  overextended  joints,  there  is  pres- 
ent in  general,  a  decrease  in  the  tonus  of  the  muscles;  if  they  lie  rigid  in 
more  or  less  forced  positions,  and  no  joint  fixations  can  be  determined,  it  is 
3 


34 


GEXERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 


usually  a  matter  of  increase  in  tonus.  The  former  condition  is  known  as 
hypotonia,  the  latter  as  hypertonia,  of  the  musculature.  Still  more  obvious 
is  the  difference  in  testing  the  voluntary,  active,  and  especially  in  testing  the 
passive  movements.  One  instructs  the  patient  to  relax  his  limbs  as  much  as 
possible  and  then  places  the  different  parts  of  the  extremities  now  with 
quicker,  now  with  slower,  now  with  brusque,  now  with  gentle,  passive 
motions  into  all  physiologically  possible  positions.  Nearly  always  one  feels 
resistance  at  first,  which  must  be  interpreted  in  part  as  reflex  increase  in  the 

normal  tonus,  and  in  part  is 
actively,  though  uncon- 
sciously evoked  by  the  pa- 
tient. But,  as  a  rule,  this 
resistance  soon  ceases  in  the 
healthy  individual.  Xow 
sometimes,  precisely  in  cases 
of  hypertonia,  this  decrease 
does  not  take  place;  nay 
rather,  at  every  attempt  at  a 
brusque  motion,  the  antag- 
onists, that  is,  those  muscles 
of  the  patient,  the  activity 
of  which  is  to  be  suppressed 
for  the  execution  of  the  in- 
tended passive  motion,  easily 
become  very  tense  and  afford 
an  almost  insuperable  resist- 
ance to  it.  On  the  other 
hand,  the  resistance  may  fre- 
quently be  overcome  by 
slower,  more  careful  motions, 
but  now  frequently  a  second 
pathological  condition  ap- 
pears: an  involuntary  spas- 
modic working  of  the  antagonists  (in  the  sense  of  the  voluntary  motion),  which 
now  suddenly,  apparently  spontaneously,  sluggishly  indeed,  but  extensively, 
sets  in  motion  the  extremity  which  before  lay  in  immobile  rigidity.  These 
symptoms  of  hypertonia  belong  to  the  large  group  of  spastic  conditions,  to 
which  we  have  to  reckon  also  the  increase  of  the  tendon  reflexes,  of  which  we 
shall  speak  in  our  discussion  of  reflexes.  In  spite  of  the  often,  as  has  been 
said,  almost  invincible,  tension  of  the  musculature  in  most  spastic  conditions, 
its  active  strength  is  loitered;  the  so-called  spastic  paresis  occurs.  As  a  conse- 
quence of  lasting  spastic  paresis,  we  find  permanent  tonic  conditions  of  the 
musculature,  contractures,  nearly  always  accompanied  by  stiffening  in  the 


Fig.  6. — Spastic  paralysis  (Little's  disease) . 


HYPOTONIA  35 

regional  joints.  These  contractures  of  the  tense,  active  (though  occasionally 
also  actively  paretic)  musculature  are  theoretically  (this  is  more  difficult  in 
practice)  to  be  distinguished  from  paralytic  contracture,  in  which  it  is  a 
question  of  mere  preponderance  of  the  strength  of  the  antagonists  as  op- 
posed to  the  paralyzed  agonists  that  have  lost  their  functional  ability  (Fig.  6). 

Decrease  of  muscular  tonus,  hypotonia,  is  found  especially  where 
the  stimulations  from  the  periphery  to  the  "power  station"  (cf.  above)  of  the 
anterior  horn  are  more  or  less  completely  lost,  that  is  in  lesion  of  the  sensory 
tracts  above  all.  Here  passive  motions  may  be  performed  with  abnormal 
ease;  the  limbs  as  a  result  of  the  relaxation  of  the  muscles  (usually  also  of  the 
articular  ligaments)  may  be  brought  into  the  most  exaggerated,  distorted  posi- 
tions. The  knee  joints  are  overextended  in  walking  (genu  curvatum),  etc. 
We  see  the  most  severe  forms  of  hypotonia  in  tabes  dorsalis.  At  the  same 
time,  the  motor  strength  may  be  altogether  normal.  The  tendon  reflexes  are 
often  simultaneously  (but  by  no  means  always)  weakened  or  absent.  Here 
belongs  the  remarkable  affection,  first  described  by  Oppenheim,  later  also  by 
Bernhardt  and  others  under  the  name  of  myatonia:  in  early  infancy,  there 
appears  an  abnormal  flaccidity  of  the  entire  musculature,  a  high  degree  of 
hypotonia,  while  at  the  same  time,  direct  and  indirect  faradic  excitability  is 
absent.  The  condition  probably  represents  a  retarded  development  of  the 
central  nervous  system  and  can  be  cured. 

If  we  return  again  to  the  test  for  active  motility,  we  see  frequently  in 
nervous  patients  a  disturbance  which  is  not  evinced  in  a  decrease  of  strength 
or  volume,  but  in  the  incorrectness  of  the  voluntary  movement:  the  move- 
ment becomes  inco-ordinated;  we  have  a  disturbance  of  co-ordination  before  us. 

The  concept  of  the  co-ordination  disturbance  is  to  be  defined  as  follows : 
In  every  movement  of  our  body  (with  very  few  exceptions)  several  muscles, 
not  one  alone,  are  synchronously  set  in  action.  The  performance  of  a 
direct  movement  is,  therefore,  an  extraordinarily  delicate  co-operation  of  these 
muscles,  known  as  synergists;  if  only  one  muscle  misses  the  cue,  so  to  say 
through  too  slight  or  excessive  contraction,  the  entire  movement  is  disturbed. 
Still  more  complicated  is  the  process,  if  (with  the  majority  of  authors)  one 
believes  that  the  antagonists  co-operate  in  every  voluntary  movement  of  the 
agonists,  whether  it  be  through  a  particularly  delicate  gradation  of  their 
relaxation  or  in  some  other  way.  If  now  for  some  reason  the  function  of 
one  muscle  is  lost,  the  others  naturally  preponderate;  the  part  of  the  body 
concerned  is  then — possibly  only  for  the  duration  of  a  fractional  part  of  the 
entire  movement— moved  in  a  direction  other  than  the  one  desired,  until 
a  new  impulse  sent  to  a  muscle,  serving  as  a  corrective,  pulls  it  back  again 
into  the  old  direction.  Thus  there  arises  a  zig-zag  movement,  a  deviation 
or  throwing  about  of  the  extremity  concerned;  we  describe  this  movement 
as  inco-ordinated  or  ataxic.  This  disturbance  is  more  or  less  independent 
of  the  will  of  the  patient,  but  may  be  'somewhat  set  aside  through  practice, 


36  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

and  often  occurs  but  slightly  in  a  patient  who  controls  with  his  eye  all  his 
movements,  and  at  every  deviation  "at  once"  calls  into  play  corrective 
muscles,  but  always  increases,  if  one  tells  the  patient  to  close  his  eyes,  and 
thereby  removes  the  "eye-control." 

The  test  of  ataxia  is  therefore  made  as  follows  in  the  individual  case. 
First  the  patient  in  a  position  of  supination  is  asked  to  make  complicated 
movements  with  his  hands:  put  his  forefinger  to  his  nose;  thread  a  needle; 
perform  the  so-called  "finger-tip"  experiment  (the  arms  which  have  been 
drawn  back  far  laterally,  are  brought  together  slowly  with  extended  fore- 
fingers until  they  touch).  When  doing  these  acts  there  appear  very  distinct 
deviating  movements,  which  produce  quite  irregular  varying  twitching 
excursions,  in  various  planes  about  the  axis  of  the  movement  desired  (in 
contrast  to  the  regulated  movements  of  most  kinds  of  tremor),  but  the 
patient,  with  the  assistance  of  the  controlling  eye,  is  usually  still  able  to 
reach  the  point  aimed  at.  If  no  clear  ataxia  has  been  shown  in  this,  one 
has  the  patient  perform  the  same  movement  after  closing  his  eyes,  whereby 
the  ataxia  usually  increases  considerably.  To  test  the  legs  in  ataxia  one 
makes  use  of  the  so-called  "knee-heel  experiment" :  the  patient  lying  down 
is  asked  to  touch  with  the  heel  of  one  foot  the  knee  of  the  other  leg,  by 
moving  the  leg  through  the  air  (not  by  drawing  it  up  along  the  recumbent 
leg).  Then  one  should  have  him  form  letters,  figures  (three)  or  draw 
circles  in  the  air  with  the  extended  leg.  All  these  movements  should  be 
repeated  with  closed  eyes.  An  ataxia  of  the  facial  muscles,  too,  may 
occasionally  be  established  by  corresponding  tests.  Then  the  patient  is 
to  sit  up.  Here  there  frequently  appears  in  a  quiet  sitting  posture  swaying 
of  the  upper  part  of  the  body  (which  still  increases  upon  standing),  com- 
parable possibly  to  a  tree  shaken  by  the  wind,  which  also  increases  when 
the  eyes  are  closed  (static  ataxia).  Now  one  should  have  the  patient  rise 
and  place  his  heels  and  toes  close  together.  Very  many  ataxics  fail  even 
in  this  movement,  they  begin  to  totter  and  have  to  put  one  foot  on  the  side, 
so  as  not  to  fall;  the  disturbance  becomes  clearer  if  one  has  the  patient 
stand  on  one  foot — a  thing  that  a  normal  person  can  do  easily  after  some 
slight  preliminary  swaying — or  specially,  if,  when  standing  with  his  feet 
close  together,  he  is  asked  to  close  his  eyes.  This  uncommonly  characteristic 
symptom  (Romberg's  phenomenon)  is  one  of  the  most  delicate  and  most 
easily  performed  tests  of  co-ordination,  and  the  manner  of  the  response 
should  be  remarked,  as  it  is  somewhat  different  in  the  various  forms  of  ataxia. 

In  the  ataxia  of  tabetics  (the  most  common  variety),  the  patient  first 
attempts  to  struggle  against  the  swaying  by  means  of  slight  movements 
with  his  feet;  then  he  places  his  feet  somewhat  apart,  and  finally  opens  his 
eyes.  In  cerebellar  ataxia  (cf.  below)  the  swaying,  already  in  itself  of  high 
degree,  does  not  increase  when  the  eyes  are  closed.  In  the  ataxia  of  hysterics 
(especially  in  traumatic  hysteria)  the  swaying  becomes  noticeable  only  when 


ATAXIA,  TREMOR  37 

the  eyes  are  closed,  but  then  immediately  becomes  excessive;  the  patients 
make  no  attempt  to  arrest  the  interesting  disturbance  and  fall  with  apparent 
carelessness  (but  in  reality  with  great  caution)  to  the  ground. 

In  walking,  ataxia,  as  a  rule,  does  not  become  higher  in  degree,  than  in 
standing,  but  it  assumes  peculiar  forms.  The  ataxic  tabetic  walks  (in  the 
most  severe  cases)  with  legs  far  apart,  protects  himself,  so  to  speak,  by 
planting  his  feet  as  widely  apart  as  possible  (to  overcome  the  swaying  that 
seizes  him)  like  a  sailor  on  ship-board.  He  likes  to  use  a  cane,  which  he 
puts  down  as  far  as  possible  from  him,  in  order  to  gain  as  large  a  triangle 
as  possible  to  serve  as  a  firm  support.  Other  tabetics  maintain  the 
direction  of  their  movements,  but  raise  their  legs  (usually  in  extension) 
unnecessarily  high,  and  replace  them  with  stamping,  a  kind  of  walk,  recal- 
ling the  older  forms  of  the  German  military  goose-step,  usually  called 
"swinging"  gait.  The  "cerebellar  ataxic"  walks  quite  differently.  He 
totters  like  a  drunken  man;  the  working  of  the  synergisms  necessary  for 
walking  is  normal,  but  the  movements  are  wrongly  connected  with  one 
another;  the  gait  looks  as  if  the  patient  had  been  suddenly  seized  with 
vertigo  (which,  as  a  matter  of  fact,  is  often  connected  with  this  form  of 
ataxia).  To  the  group  of  cerebellar  ataxia  belongs  also  the  disturbance 
described  by  Babinski  as  "  asynergia,"  which  occasionally  appears  unilater- 
ally. x\taxic  tabetics  walk,  as  a  rule,  far  more  poorly  in  the  dark  than  by 
day;  this  statement  also  is  often  characteristic,  that  their  ataxia  had  been 
noticed  first  by  them  in  the  morning  when  they  had  closed  their  eyes  to 
wash  the  face. 

The  regulation  essential  for  co-ordination  of  movements  is  mediated  by 
centripetal — in  the  main,  therefore- — sensory  stimulations,  which  become 
transformed  in  the  centers  of  the  cerebrum  and  the  cerebellum;  it  is  not  yet 
known  how  far  the  system  of  semicircular  canals  in  the  labyrinth,  which 
unquestionably  serves  to  maintain  equilibrium,  subserves  co-ordination. 
Disturbances  in  co-ordination,  ataxia,  will  appear,  therefore,  as  soon  as  the 
paths  or  centers  of  regulation  are  interrupted:  tabes,  Friedreich' s  disease  and 
possibly  ataxic  polyneuritis  on  the  one  hand,  affections  of  the  cerebellum  and 
(very  rarely)  those  of  the  cerebrum,  on  the  other,  lead  to  ataxia,  but,  strange 
to  say,  never  mere  affections  of  the  centrifugal  path — that  is,  pure  motor 
paralyses  with  exception  of  the  rare  polyneuritic  ataxia.  We  must,  more- 
over, here  repeat  again  that  ataxia  has  nothing  to  do  with  disturbances  in 
coarse  strength,  though  occasionally,  motor  conditions  of  weakness  other 
than  ataxia  may  occur  (ataxic  polyneuritis). 

Ataxia,  to  a  certain  extent,  represents  an  "  insufficiency "  of  irritation 
phenomena  of  the  motor  sphere,  combined  with  unregulated  motor  in- 
nervation. We  come  now  to  several  symptomatic  groups,  all  of  which  are 
characterized  by  an  "excess"  of  motor  irritation  phenomena. 

As    tremor   are   designated    regular   involuntary    movements    following 


38  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

one  another  rapidly,  taking  place  in  smaller  or  larger  excursions  about  an 
axis  and  always  in  one  plane;  they  are  evoked  by  involuntary  muscular  con- 
tractions, in  which  agonists  and  antagonists  alternately  participate.  One 
distinguishes  between  a  fine  and  a  coarse  tremor,  according  to  the  size  of  the 
excursions,  eventually  also  between  a  rapidly  and  a  slowly  oscillating  tremor. 
Furthermore  there  must  be  differentiated,  principally,  a  tremor  which  appears 
only  upon  motion,  and  one  that  appears  only  in  rest.  The  two  extremes  are 
represented  by  the  tremor  of  multiple  sclerosis  and  that  of  paralysis  agitans. 
The  sufferer  from  multiple  sclerosis  in  a  state  of  rest,  is,  as  a  rule,  abso- 
lutely free  from  tremor.  Also  in  simple  tonic  innervation,  if,  for  instance,  he 
holds  his  hands  quietly  extended,  no  tremor  appears.  As  soon,  however, 
as  he  intends  to  perform  complicated  movements,  as  in  the  test  in  which  an 


I  egivving 


Beginning     ..ill/! J ' l\ /! 'J /  I i 


JlJlJ/fli"'  Left  Hand  Right  Hand    ■• 

Fig.  7. — Tracing  of  the  intention  tremor  in  multiple  sclerosis. 

attempt  is  made  to  bring  together  the  tips  of  his  fingers,  tremor  appears. 
This  tremor  is  first  characterized  by  small  excursions,  but  the  more  the 
movement  comes  "to  a  point,"  the  more  delicate  it  becomes,  the  greater 
the  demands  to  reach  the  desired  point,  just  as  in  the  finger-tip  experiment, 
at  the  moment  the  finger  tips  meet,  the  larger  the  excursions  become, 
which  can  be  represented  by  a  diagram  as  follows  (Fig.  7) : 

If  one  has  an  ataxic  perform  the  same  movement,  the  diagram  will  be 
something  like  the  following  (Fig.  8) — in  which  one  must  consider,  more- 
over, that  the  deviating  excursions  of  ataxia  may  lie  in  altogether  different 
planes. 


vV-A/^ 


Fig.  8. — Tracing  of  intention  ataxia  in  tabes.     The  individual  strokes  of  the  line  frequently  lie  in 

quite  different  planes. 

It  is,  therefore,  certainly  not  permissible  to  confound  the  "intention 
tremor"  of  multiple  sclerosis,  with  the  "intention  ataxia''''  say,  of  tabetics, 
though  there  may  be  some  points  of  similarity.  Even  the  innervation  dis- 
turbances upon  which  they  depend  may  be  different. 

Quite  different  is  the  tremor  of  paralysis,  agitans  {Parkinson  s  disease). 
Here  in  intended,  voluntary  movements,  there  is  little  or  no  tremor;  but  if 
the  patient  allows  his  extremities  to  lie  or  hang  quietly,  if  he  places  his 
hands  in  his  lap,  etc.,  there  appears  usually  a  very  fine  tremor  with  rapid 
oscillations,  which,  in  severe  cases,  persists  in  sleep  and  in  the  beginning 
often  attacks  only  one  extremity,  then,  perhaps,  half  of  the  body,  finally, 


NYSTAGMUS,  ASSOCIATED  MOVEMENTS  39 

usually  the  entire  body.  It  also  appears  in  the  hands  in  a  peculiar  type  of 
tremor, designated  as  "coin-counting"  (pill-rolling),  which  consists  of  move- 
ments of  the  thumb  in  front  of  the  other  fingers — that  recall  the  movement 
from  which  it  derives  its  name. 

Most  other  forms  of  tremor  are  of  the  type  of  fine  oscillations,  usually 
only  slight  but  nevertheless  present  in  the  state  of  rest,  increasing  upon 
motion,  such  as  neurasthenic  and  hysteric  tremor,  alcoholic  and  senile  tremor 
and  the  tremor  in  Basedow's  disease.  Slight  tremor  can  be  made  plainly 
visible  by  laying  a  piece  of  paper  on  the  outstretched  fingers  of  the  patient. 

Nystagmus  represents,  as  has  been  mentioned  above,  an  "intention 
tremor"  of  the  eye  muscles.  Some  forms  of  tonic  spasm,  as,  for  instance, 
blepharospasm  (eye-lid  spasm)  of  hysterics,  are  also  associated  with  slight, 
finely  oscillating,  tremor  of  the  muscles  concerned. 

The  so-called  "fibrillary"  or  u fascicular'"  twitchings  of  parts  of  one 
muscle  represent  a  totally  different  process.  In  this,  we  see  at  various  places 
on  the  surface  of  the  body,  small  twitchings  of  individual  bundles  of  muscles, 
usually  taking  place  without  any  effect  on  movement;  they  are  occasionally 
rhythmical,  recalling,  then,  especially  shivering  from  cold,  but  are  frequently 
arythmical,  attacking  always  different  muscular  bundles  in  the  same  region 
and  may  lead  to  a  total  "muscular  wave"  {myokymia).  The  genuine 
fibrillary,  that  is,  smallest,  muscular  twitchings  of  this  kind  are  found  only 
in  organic  diseases  of  the  central  nervous  system.  All  other  muscular  groups 
can,  after  sufficient  practice,  be  distinguished  from  these.  We  must  add 
here,  moreover,  that  in  affections  of  the  anterior  horn,  etc.,  which  are  asso- 
ciated with  muscular  atrophy,  these  twitchings  are  restricted,  in  the  main, 
to  the  atrophic  areas,  and  occasionally  also  indicate  the  spread  of  the 
process  to  other  muscular  parts  preceding  the  atrophy. 

In  the  realm  of  involuntary  movements,  there  belong  the  associated  or 
co -movements.  Small  children  scarcely  ever  make  movements  of  grasping,  etc., 
with  one  hand  alone,  but  innervate  both  hands  and  arms  synchronously; 
complicated  processes,  such  as  crying  and  laughing,  are  also  very  frequently 
found  together  with  associated  movements  on  the  part  of  the  extremities. 
These  co-movements,  with  the  progress  of  development  disappear,  leaving 
behind  only  traces,  but  are  occasionally  found  even  at  a  more  advanced  age, 
and  return  in  pronounced  fashion  in  organic  paralyses,  especially  in  those  of 
cerebral  origin.  Most  hemiplegics,  for  instance,  can  not  make  energetic 
movements  of  the  sound  arm  entirely  without  associated  movements  of  the 
paralyzed  arm,  and  this  symptom  is  an  important  differential  diagnostic 
criterion  between  organic  and  psychogenous,  hysteric  paralyses,  in  which 
latter  it  is  always  lacking  {Hans  Curschmann). 

Finally  the  last  three  groups  of.  involuntary  hyperkinesis,  those  which  we 
designate  choreic  disturbance  in  movement,  tic  and  athetosis,  show  a  con- 
siderable similarity  to  one  another.     Each  is  well  characterized  in  itself, 


4Q 


GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 


presents  a  peculiar  form  of  disease,  may  often  be  classed  among  the  neuroses, 
and  may  therefore  occur  without  any  "  organic  symptoms."  Therefore  their 
knowledge  and  recognition  are  particularly  important.  Chorea  is  character- 
ized by  sudden  violent  twitching  excursions,  without  rhythm  and  regularity, 
appearing  in  rest,  and  not  totally  disappearing  upon  motion.  In  these 
movements  single  muscles  as  well  as  muscular  groups  and  entire  extremities 
are  involved;  in  the  general  impression  the  "restlessness  of  the  limbs" 
predominates,  since  some  of  the  violent  movements  may  seem  absolutely 
intentional.  The  patient  puts  his  hand  up  to  his  nose,  purses  his  lips  for 
whistling,  reaches  for  an  object,  etc.  Frequently  the  disease  is  first  uni- 
lateral, but  usually  it  appears  over 
the  entire  body  affecting  especially, 
inmost  cases,  the  face  (making 
grimaces,  rolling  the  eyes,  etc.) — 
and  besides  this,  very  frequently 
the  psyche  also  is  affected  (Fig.  9) . 
In  the  most  severe  cases,  the 
twitchings  follow  one  another  in 
uninterrupted  succession,  the 
patient  throws  himself  about  in 
bed,  tears  his  hair,  rends  the 
sheets  and  his  clothes,  can  scarcely 
be  quieted  with  hypnotics,  and  fre- 
quently dies  in  this  condition. 
Less  severe  are  the  comparatively 
rare  choreic  movements  in  organic 
diseases  of  the  brain.  In  contrast 
to  this,  tic  represents  a  regulated 
movement,  in  so  far  that  it  always 
attacks  only  definite  groups  of 
muscles,  usually,  however,  in  the 
same  brief  twitching  way  as  in 
chorea.  The  movement  may,  at 
first  sight,  appear  voluntary,  in- 
tentional; the  patient  shrugs  his 
shoulders,  throws  his  head  backwards,  strikes  the  table  with  his  hand.  The 
same  movement  is  repeated,  not  rhythmically,  but  at  all  times,  upon  all 
occasions,  increases  especially  during  excitement,  and  can  not  be  suppressed 
in  spite  of  the  most  earnest  desire  of  the  patient  to  put  an  end  to  it.  If  the 
motility  disturbance  affects  muscular  groups  over  the  entire  body,  one  speaks 
of  "maladie  des  tics,"  which,  however,  can  not,  in  its  nature,  be  totally  identi- 
fied with  simple  tic  (Gilles  de  la  Tourette).  Chorea  and  tic  are  frequently 
accompanied  by  psychic  disturbances.     Athetosis  also  occasionally  occurs  as 


FlG.'o 


-Clonic   spasms     in    chorea    hereditaria. 

{After  Huntington.) 


SPASMS,  CONVULSIONS  41 

an  independent  neurosis,  sometimes,  however,  after  hemiplegia  in  the  para- 
lyzed limbs,  is  frequently  combined  with  contractures  and  nearly  always 
affects  only  fingers  or  toes.  In  this  case,  the  movements  are  sluggish, 
vermicular,  always  with  considerable  effect  upon  motility  and  often  of  very 
strange  form.  The  fingers  are  extended,  stretched,  overstretched  and  bent 
in  the  most  remarkable  manner,  assuming  the  most  unlikely  positions. 
Whereas  in  chorea  and  especially  in  tic  there  are  usually  longer  or  shorter 
intervals  of  rest  between  the  separate  movements,  athetosis  is  an  almost  un- 
ceasing hyperkinesis,  suppressed,  as  a  rule,  only  during  sleep. 

Spasms  (cramps)  have  often  been  designated  as  hyperkinesis  in  the 
narrower  sense,  among  which  we  can  with  some  justification  reckon  the 
motility  disturbances  we  have  just  mentioned  (especially  tic).  We  dis- 
tinguish as  main  groups,  tonic  and  clonic  spasms.  By  tonic  spasm  we  mean 
permanent  conditions  of  contraction  in  one  muscle  group;  the  most  severe 
forms  are  called  tetanic  spasm,  tetany.  Clonic  spasms  are  brief,  rapid 
twitchings,  usually  repeating  themselves  with  a  certain  rhythm,  which  may, 
in  the  highest  degree,  attack  the  entire  body  (convulsions) .  We  have  already 
found  an  example  of  isolated  clonic  spasm  in  tic.  It  appears  frequently  as  a 
reflex  spasm  (tic  convulsif  of  the  facial  area) .  The  majority  of  spasm  forms 
belonging  to  this  chapter,  however,  are  to  be  classed  among  the  neuroses  or 
psycho-neuroses,  and  may  all  probably  be  localized  in  the  cerebral  cortex. 
This  is  certainly  true  of  the  spasms  in  Jacksonian  epilepsy,  where,  as  the 
result  of  irritation  of  a  definite  part  of  the  cortex  (scar,  tumor)  tonic,  or  more 
frequently,  clonic  spasms,  always  of  definite  muscular  group,  usually  only 
unilateral  in  the  opposite  half  of  the  body,  are  evoked,  with  or  without  loss 
of  consciousness.  Analogously,  we  may  assume  that  convulsions  of  common 
epilepsy  may  originate  in  the  cerebral  cortex.  The  question  of  localization 
with  respect  to  the  major  hysteric  attacks  of  spasms  is  more  difficult  to  answer 
(Fig.  10).  In  appearance,  epileptic  and  hysteric  attacks  are  but  little  dif- 
ferent and  may  pass  over  into  each  other.  As  to  the  differential  diagnosis, 
more  will  be  said  in  the  special  chapters.  In  general,  the  tonic  conditions 
predominate  in  epilepsy;  usually  consciousness  is  entirely  lost.  In  hysteria, 
even  the  "major  attack"  is  usually  composed  of  periods  of  tonic  rigidity  and 
clonic  twitchings  and  consciousness  is  not  totally  lost,  at  least  a  psychic  re- 
action can  usually  be  evoked  by  means  of  external  influence,  especially  if 
painful.  The  epileptic  patient  falls  to  the  ground,  usually  without  noticeable 
prodromes  (aura)  of  the  attack,  his  arms  and  feet  twitch  a  little,  his  teeth 
gnash  spasmodically,  and  there  soon  appears  a  high  degree  of  tonic  tension 
throughout  his  entire  body  with  finely  oscillating  clonic  vibrations  of  the 
muscles  in  tension;  respiration  becomes  superficial,  the  patient  wheezes, 
becomes  cyanotic,  foams  at  the  mouth,  frequently  urine  is  voided  involuntar- 
ily, until  after  a  few  minutes  (rarely  more) ,  tension  is  relaxed  and  conscious- 
ness as  a  rule,  soon  returns  with  the  restoration  of  the  reflexes  that  had  dis- 


42 


GEXERAL  DIAGNOSTICS  OF  XERVOUS  DISEASES, 


appeared  (also  of  the  pupils).  The  entire  event  creates  an  impression  of  a 
real,  genuine  attack;  not  rarely  the  patients  are  injured  by  falling,  or  bite 
their  tongues.  In  the  hysteric  attack  half  is  usually  "histrionic."  The 
attack  rarely  appears  when  the  patients  are  wholly  unobserved;  frequently  it 
crowns  the  demonstration  of  a  passionate  emotional  outbreak.  The  hyster- 
ical patient  produces  his  effects  with  far  coarser  means  than  the  epileptic; 
the  entire  body  is  convulsed  with  twitchings,  assumes  "attitudes  passionelles," 
like  the  position  of  a  crucified  man,  erotic  positions,  etc.,  lies  in  bed  in  the 
form  of  an  arch,  so  that  only  the  back  of  the  head  and  the  heels  touch  the 


Fig.  io. — Hysteria  (major  hysteric  attack).     Attention  is  called  to  the  opened  and  fixed  eyes. 
(After  Schoenborn-Krieger ,  Klinischer  Atlas  der  Nervenkrankheiten) 


sheet.  Any  suggestive  influence,  pressure  in  "  hysterogenous "  zones, 
faradization,  in  lighter  cases,  sprinkling  with  cold  water  and  addressing  the 
patient  may  check  the  attack,  which  otherwise  may  last  for  hours.  If  the 
patient  falls  to  the  ground,  he  usually  falls  carefully  so  as  not  to  injure  him- 
self. In  spite  of  all  these  signs  of  recognition,  the  differentiation  of  these  two 
forms  of  spasm  may,  as  has  been  said,  be  very  difficult.  For  a  discussion  of 
separate  rarer  forms  of  spasms,  such  as  the  tonic  tetanic  spasms  which  are 
localized  especially  in  the  extremities,  myoclonus,  occupational  cramps,  as 
well  as  for  traumatic  tetanus,  cf.  the  particular  divisions  devoted  to  these 
subjects. 

In  the  test  of  motility  or  of  coarse  strength  we  have  still  two  phenomena  to 
mention.  In  some  cases,  upon  first  testing  the  muscles,  as  in  warmly 
shaking  the  patient's  hand  to  see  how  strong  his  grip  is,  etc.,  his  strength  is 


MECHANICAL  MUSCULAR  EXCITABILITY  43 

very  good,  but  he  is  unable  to  relax  the  muscle  that  has  been  contracted. 
But  if  he  repeats  the  same  movement  several  times  in  succession,  "for 
practice,"  the  contraction  and  relaxation  take  place  more  and  more  rapidly, 
finally,  at  the  normal  rate  of  speed;  the  grasp  of  the  hand,  which,  like  the 
clutch  of  a  vise,  would  not,  in  spite  of  all  the  endeavors  on  the  part  of  the 
patient,  release  the  examiner's  hand,  can  now  no  longer  be  distinguished  from 
the  grasp  of  a  healthy  man,  until  after  some  period  of  rest,  the  first  condition 
reappears.  In  this  disease,  myotonia  (Thomsen's  disease),  which,  as  a  rule, 
attacks  the  entire  body  and  may  be  accompanied  by  very  good  coarse 
strength,  even  with  hypertrophic  musculature  (cases  of  atrophy  are  rare), 
abnormal  anatomic  conditions  and  transformations  in  the  muscle  itself 
probably  play  the  main  serological  role.  In  a  certain  sense,  myasthenia  or 
myasthenic  paralysis  forms  a  contrast  to  this — the  picture  of  an  abnormal 
tendency  to  fatigue  of  the  otherwise  apparently  normal  musculature.  Here, 
it  is  true,  the  patient  at  first,  for  a  few  times,  may  perform  the  prescribed 
movement  rapidly  and  powerfully;  after  the  fifth  or  tenth  time  his  strength 
decreases,  and  soon,  in  spite  of  all  his  efforts,  he  is  unable  to  execute  the 
movement,  until,  after  a  resting  period,  the  muscles  have  again  recovered. 
For  this  disease  picture,  which  has  not  yet  been  consistently  interpreted, 
there  exist,  moreover,  as  for  myotonia,  analogous  electrical  changes  (cf. 
below). 

An  absolutely  essential  part  of  every  test  of  the  motor  sphere  is  the 
electrical  examination.  But  it  should  be  preceded  by  the  test  of  the  mechani- 
cal excitability  of  the  muscles  and  nerves,  comparable  with  it  in  many  respects. 
We  understand  by  this,  naturally,  not  the  pain  stimulations  that  affect  the 
skin,  but  the  movements  due  to  pressure,  tapping  or  striking,  which  affect 
the  skin  as  little  as  possible,  and  strike  the  underlying  portions,  as  far  as 
possible,  in  isolation.  For  this  test,  almost  without  exception,  only  the  finger 
of  the  examiner  or  the  tap  of  the  percussion  hammer  is  suitable. 

Changes  in  mechanical  muscular  excitability  are  demonstrable  almost 
exclusively  in  the  form  of  an  increase  in  excitability,  or  of  qualitative  change 
in  it.  For  there  is  no  norm  in  respect  to  the  effect  of  tapping  a  sound  muscle. 
The  local  muscular  prominence  may  form  an  " idiomuscular  contraction" 
but  it  may  be  very  slight  and,  as  happens  in  the  majority  of  cases,  may 
disappear  again  immediately;  it  may  also  be  very  pronounced  and  persist 
for  some  time.  This  increase  in  mechanical  muscular  excitability  is  found  in 
tuberculosis,  typhoid,  in  most  cachexias,  here  and  there,  in  sciatica  and 
affections  of  the  anterior  horn.  Very  frequent,  and  occasionally  classical,  on 
the  other  hand,  is  the  qualitative  change  in  excitability,  the  mechanical  proof 
of  the  reaction  of  degeneration  (cf.  below),  or  more  correctly  the  "sluggish 
mechanical  contraction"  as  it  is  often  found  in  the  degenerative  atrophies, 
especially  at  an  early  stage.  In  ulnaris  neuritis,  bulbar  paralysis,  etc., 
tapping  the  ball  of  the  little  finger,  or  the  thumb,  evoke  the  most  beautiful 


44 


GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 


"sluggish  contractions."  In  Thomsen's  disease,  also,  the  mechanical  tonic 
twitching  corresponding  to  the  myotonic  reaction  (cf.  below),  the  "arrest" 
of  contraction  may  be  typically  demonstrated.  The  theoretically  postulated 
lowering  of  mechanical  excitability  for  severe  old  paralyses,  for  myasthenia, 
etc.,  can  not,  on  the  other  hand,  as  a  rule,  be  shown.  For  the  mechanical 
excitability  of  the  nerves  also — which,  in  our  sense,  can  be  used  only  for  motor 
nerves — only  an  increase  in  excitability  can  be  demonstrated;  decrease  and 
qualitative  change  can  not  be  proved.  The  mechanical  increase  in  the 
excitability  of  the  nerves  is  best  shown  in  some  nerves  lying  near  the  surface : 


Frontalis. 
Facial  {upper). 

Corrugator    super- 
cilii. 

Orbic.      palpe- 
brarum. 

Nasal    muscles.  -J 

Zygomatici. 

Orbic.  oris,  -j 

Facial  (middle) . 
Masseter. 

Levator  menti. 

Quadratus. 

Triangularis. 

Hyfoglossus. 

Facial  (lower). 

Platysma  myoides. 

Hyoid  muscles.  ] 

Omohyoid. 


Ext.  anterior, 
thoracic  (pectoralis 
major). 


Ascending  frontal  and 
parietal  convolutions 
(motor  area). 


Third  frontal  convolu- 
tion and  insula  (cen- 
ter of  speech). 

Temporalis. 
Facial  (upper  branch). 
Facial  (trunk). 
Posterior  auricular. 
Facial  (middle  branch). 
Facial  (lower  branch). 
Splenius. 

Sternomastoideus. 

Spinal  accessory. 
Levator   anguli 

scapulae. 
Trapezius. 

Dorsalis   scapula 
(rhomboidei). 


Circumflex. 


Long-     thoracic     (ser- 
ratus  magnus). 


Phrenic.        Fifth  and  sixth  cervical 
(deltoid,  biceps,  bra- 
chialis  anticus,  supi- 
nator longus). 


Brachial  plexus. 


Fig.  ii. — Motor  points  of  face  and  neck.     {After  Erb  and  de  Watterville.) 

ulnaris  (at  the  elbow),  peroneus  (capit.  fibulae),  facialis  (most  of  its  branches 
as  well  as  the  trunk) .  This  increase,  as  a  matter  of  fact,  is  found  only  in  a 
single  disease,  tetany,  in  which  tapping  upon  the  ulnaris  at  once  evokes 
twitchings  in  the  muscles  of  the  ball  of  the  little  finger,  tapping  or  stroking 
of  the  facial  branches,  unilateral  twitching  of  the  mimetic  muscles  (Chvostek's 
symptom) . 

The  conclusions  reached  by  testing  the  electrical  reaction  of  the  muscles 
and  nerves  are  very  much  more  comprehensive.  But  in  this  field,  which  in 
modern  medicine,  even  in  neurology,  is  somewhat  unkindly  treated  and 


ELECTRICAL  EXCITABILITY 


45 


rather  neglected,  there  are  necessary,  more  than  in  others,  careful  observa- 
tion, experience  and  detailed  knowledge  of  the  technique  (above  all  of  the 
electrical  apparatus  used  by  the  examiner) .  Therefore,  if  possible,  one  should 
make  an  electrical  examination  of  patients,  only  with  an  apparatus  one  owns 


Triceps  (long  head). 

Triceps  (inner  head). 
Ulnar. 


Flexor  carpi  ulnaris. 

Flexor  profundus 
digitorum. 


Flexor  sublimis  digitor 
um  (II  and  III). 


Flexor  sublimis  digitor- 
um (index  and  little 
fingers). 

Ulna?-. 


Palmaris  brevis. 

Abductor  min.  digit. 

Flexor  min.  digit. 

Opponens  min.  digit. 


I/umbricales.  - 


Deltoid     (anterior 
portion). 


sublimis  digitorum. 
Flexor  longus  pollicis. 
Median. 

Abductor   pollicis. 
Opponens  pollicis. 
Flexor  brevis  pollicis. 
Adductor  pollicis. 


Fig.  12. — Motor  points  on  upper  limb,  flexor  surface.     {After  Erb  and  de  Watterville.) 


oneself,  or,  at  least,  always  with  the  same  kind,  which  should  comprise  a 
faradic  (with  the  greatest  possible  variation  in  the  relation  between  tube  and 
core)  and  a  galvanic  battery  with  a  good  galvanometer,  as  well  as  several 
ordinary  electrodes,  a  button  electrode  and  several  plate  electrodes  (surface 
of  10  to  60  sq.  cm.). 


46 


GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 


We  work,  as  a  rule,  with  two  electrodes  of  different  size,  a  large  so-called 
indifferent  one,  about  60  sq.  cm.  and  a  small  active  one,  as  like  as  possible 
Erb's  normal  electrode  of  10  sq.  cm.  (round  or  square).  The  large  one  is 
placed  upon  an  indifferent  spot  (the  sternum  is  best)  and  may  be  held  there  by 
the  patient  himself;  the  small  one  is  placed  upon  the  muscles  and  nerves  to 
be  tested.     For  the  necessary  opening  and  closing  of  the  current,  one  makes 


Deltoid  (poste- 
rior part). 


Muscidospi7-al. 
Brachialis   anticus. 

Supinator  longus. 
Ext.  carpi  radial,  longior. 
Ext.  carpi  radial,  brevior. 


Extensor  communis  ( 
digitorum.  \ 


Extensor  indicis 


Ext.  ossis  metacarpi   pollicis. 
Ext.  primi  internodii  pollicis. 


t     T  f   fl  » 

Dorsal  interossei.  <  tj      ' 


:-*    Triceps  (long  head). 


Triceps  (outer  head)- 


Extensor  carpi  ulnaris. 
Supinator  brevis. 

Extensor  minimi  digiti. 
Extensor  indicis. 

Extensor   secundi    internodii 
pollicis. 


Abductor  minimi  digiti. 
Dorsal  interossei  (III  and  IV). 


Fig.  13. — Motor  points  on  upper  limb,  extensor  surface.     (After  Erb  and  de  Watterville.) 

use  of  the  interrupting  electrode,  upon  the  handle  of  which,  pressure  on  a 
button  or  a  smiliar  device,  causes  a  break  in  the  current. 

Now,  which  points  does  one  examine?  Where  does  one  set  the  active 
electrode?  In  general,  upon  the  empirically  determined  points  of  entry  of 
the  nerves  into  the  muscles — for  the  electric  test  of  the  musculature — and 
upon  the  places  in  the  nerve  trunks  where  these  approach  the  surface.  These 
"muscle  and  nerve  points"  were  determined  by  Erb  upon  a  foundation  of 


ELECTRICAL  EXCITABILITY 


47 


extensive  and  detailed  investigation  (Figs,  n  to  16).  The  significance  of  the 
individual  points  is  clear,  without  further  discussion ;  they  show  that  if  one 
places  the  large  electrode  upon  the  sternum  and  the  small  one  upon  one  of 
the  points  upon  the  surface  of  the  body  here  designated  and  employs  (farad- 
ically)  a  sufficiently  strong  current  or  (galvanically)  opens  and  closes  the 
current,  then,  the  muscle  named  in  the  tables  (at  the  nerve  points,  the  muscles 
subserved  by  this  nerve)  twitches,  that  is,  momentarily  (galvanically)  or 
tonically,  lastingly  (faradically)  contracts.  If  it  be  a  matter  of  a  diffuse 
affection,  all  these  muscular  and  nerve  points  must  be  examined  in  this  way, 


Anterior  crural. 

Obturator. 

Pectineus, 

Adductor  magnus. 
Adductor  longus, 


Crureus 


Vastus  internus 


\  Tensor  fasciae  femoris 

Sartorius. 

Quadriceps  femoris. 
Rectus  femoris. 

Vastus  externus 


Fig.  14. — Motor  points  on  thigh,  anterior  surface.     {After  Erb  and  de  W.attervitte.) 


while  in  local  affections  only  the  regionary  muscular  and  nerve  points  are  to 
be  thus  examined.  First  the  faradic  and  then  the  galvanic  examination 
should  be  made. 

In  testing  the  faradic  excitability,  one  determines  what  minimal  strength 
of  current  barely  suffices  to  evoke  a  contraction  of  the  muscles,  and  (theo- 
retically) in  the  indirect  test — stimulation  of  the  nerves — as  well  as  in  the  direct 
stimulation  of  the  muscles.  This  minimal  power  of  the  current  is  shown  in 
millimeters  upon  the  instrument,  but  since  all  apparatus  for  measuring  the 
faradic  current  are  unreliable,  and  not  absolutely  equal  in  any  two  sets  of 


48  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

apparatus,  the  examination  is  of  direct  value  only  where  one  can  compare  in  a 
patient  the  diseased  muscle  and  nerve  with  the  sound  one  on  the  other  side, 
when  one  can,  to  a  certain  extent,  estimate  the  capacity  of  the  skin  to  resist 
the  current  and  works  with  an  apparatus  one  has  already  tried.  Certain 
normal  medium  figures  for  these  "threshold"  values  have  been  determined 
by  Erb,  Stintzing  and  others  and  may  serve  as  points  of  reference;  usually 
Stintzing's  figures  are  used.  But  the  unskilled  examiner  (especially  when  the 
nerve  points  are,  as  is  sometimes  the  case,  hard  to  find)  often  meets  consider- 
able variations  from  these  mean  values.  Of  these  mean  values,  we  shall 
reproduce  here  some  from  Stintzing's  instrument.  But  we  must  emphasize 
the  fact,  that  in  direct  muscular  stimulation,  the  excitability  fluctuates  within 
such  wide  bounds,  that  usually  one  is  contented  with  reproducing  the  most 
manageable  values  of  the  limit  of  indirect  (nerve-)  excitability. 

Distance  table  for 
Nerve  or  muscle  minimal  twitching 

N.  facialis 132-110 

N.  medianus 135-110 

N.  ulnaris 130-107 

N.  peroneus 127-103 

N.  cruralis 120-103 

N.  radialis 120-90 

N.  accessorius 145-130 

N.  musculocutaneus 145-125 

N.  axillaris 125—93 

N.  thoracicus  ant 145-110 

The  direction  of  the  current  is  of  no  importance  in  this  test.  In  faradic 
stimulation,  upon  interruption  of  the  current  ("opening  shocks")  there 
appears  a  momentary  contraction,  otherwise,  a  tonic  contraction  lasting  as 
long  as  the  current  is  applied. 

The  procedure  in  testing  the  galvanic  excitability  of  the  muscles  and 
nerves  is  somewhat  different.  Here,  too,  the  large  indifferent  electrode  is 
placed  upon  the  sternum  (possibly  upon  the  nape,  or  the  lumbar  portion  of 
the  spinal  column),  the  small,  active  one  upon  the  points  to  be  tested.  Only 
opening  and  closing  of  the  current  evokes  muscular  twitching,  not  the  dura- 
tion of  the  current.  The  contraction  in  the  normal  state,  is  brief,  lightning- 
like; tetanus  appears  only  upon  the  use  of  very  strong  currents — but  never 
"sluggish  contraction"  (cf.  below).  But  here  the  direction  of  the  current 
is  of  importance.  We  always  begin  by  taking  as  the  " active"  electrode, 
the  cathode  or  negative  pole. 

Now  we  determine  at  what  power  of  the  current  appears  the  minimal 


ELECTRICAL  EXAMINATION 


49 


contraction  for  direct  and  for  indirect  stimulation.  The  measure  of  the 
strength  of  the  current  is  here  given  exclusively  by  the  galvanometer,  which 
directly  indicates  in  milliamperes  the  strength  of  the  current.  It  is  best, 
in  every  examination  to  insert  the  galvanometer  from  the  first  into  the 
circuit  of  the  current,  since  the  resistance  that  it  always  offers  to  the  current, 
would,  if  it  were  put  in  and  taken  out  later,  destroy  the  result  of  the  test; 
if  the  galvanometer  is  excluded,  a  smaller  number  of  elements  will  always 
be  necessary  to  secure  the  same  strength  of  current  and  the  minimal  con- 


) 

r  Gluteus  maximus, 


Sciatic. 

Biceps  (long  head). 
Biceps  (short  head). 


Peroneal. 


Gastrocnemius 
(outer  head). 


Soleus. 


Flexor  longus  hallucis. 


) 


Adductor  magnus. 

Semitendinosus. 

Semimembranosus. 


Posterior  tibial. 


Gastrocnemius 
(inner  head). 


Soleus. 


Flexor  longus  digitorum. 
Posterior  tibial. 


Fig.  15. — Motor  points  on  lower  limb,  posterior  surface.     {After  Erb  and  de  Watterville.) 


traction.  Furthermore,  one  must  see  to  it  that  the  electrodes,  as  always 
should  be  done  in  electrical  examinations,  are  thoroughly  moistened  with 
warm  water  (salt  water  is  unnecessary),  and  remember  that  in  the  gal- 
vanic test,  the  resistance  of  the  skin  to  the  current  decreases  with  the  length 
of  application. 

For  galvanic  minimal  contractions,  too,  a  series  of  average  values  have 
been  given;  some  of  the  most  important,  according  to  Stintzing,  are: 
4 


50  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

Minimal  strength  of 
the  current  in  Milli- 
Nerve  amperes  (mean  wave) 

N.  facialis 1.0-2.5 

N.  accessorius o  .01-0 .  44 

N.  medianus 0.3  -1.5 

N.  ulnaris 0.6-2.6 

N.  radialis 0.9  -2.7 

N.  cruralis 0.4-1.7 

N.  peroneus 0.2  -2.0 

N.  tibialis  post 0.4-2.5 

The  values,  as  shown  above,  vary  considerably  even  in  sound  persons. 
But  the  following  must  be  remarked:   these  values,   and  especially  the 


Tibialis  anticus.  _, 
Extensor  longus  digitorum 


Peroneus  brevis. 


Extensor  longus  hallucis. 


Interosse 


_        Peioneal. 


Gastrocnemius. 
Peroneus  longus. 


•Soleus. 


Flexor  longus  hallucis. 


Extensor  brevis  digitorum, 
Abductor  minimi  digiti. 


Fig.  16. — Motor  points  on  leg,  external  surface.     {After  Erb  and  de  Watterville.) 

"threshold"  values  of  the  galvanic  stimulation  of  the  sound  nerves,  hold 
good  for  the  cathodal  closure  contraction  (C  C  C)  that  is,  for  the  twitching, 
which  appears  when  the  cathode  is  placed  as  active  electrode  upon  the  point 
to  be  tested,  and  the  current  is  passed  in,  then  closed  off.  According  to 
the  law  of  contraction  this  is  the  twitching  that  first  appears  in  normal  nerves. 


ELECTRICAL  EXAMINATION  51 

Now,  if  we  open  the  current,  and  then  by  means  of  the  commutator  which 
is  necessary  in  every  galvanic  battery,  reverse  it,  and  then  alternately 
interrupt  it  (opening  and  closing),  whereby  the  active  pole  which  was 
formerly  the  cathode  has  now  become  the  anode,  we  find,  if  the  power  of 
the  current  is  kept  as  strong  as  before,  no  further  twitching.  But  if  we 
slowly  increase  the  current,  we  see  first  that,  besides  the  twitching  that 
results  from  cathodal  closure  and  remains  the  strongest,  there  appears  a 
contraction  upon  opening  (interrupting)  the  current,  at  the  pole  that  has 
become  the  anode — the  anodal  opening  contraction;  at  the  same  time,  too 
(sometimes  a  little  earlier),  there  occurs  a  contraction  at  closing  the  anode 
(A  O  C  and  A  C  C) .  If  we  further  increase  the  current,  there  results  at  the 
cathode,  upon  closing  the  current,  instead  of  the  former  brief  twitching, 
tetanus  (C  C  Te),  as  long  as  the  current  is  kept  closed,  upon  still  further 
increase,  contraction  at  the  opening  of  the  cathode  (C  O  C),  and  an  anodal- 
closure  tetany  (A  C  Te).  Naturally,  it  is  theoretically  possible  and  occurs 
also  in  practice,  that  at  the  place  where  the  indifferent  electrode  is  used, 
twitchings  are  also  evoked  simultaneously  with  those  at  the  active  pole 
(occasionally  especially  disturbing  in  facialis  paralysis) .  This  disturbance, 
which  may  make  the  picture  confusing,  should  be  avoided  by  taking  a  large 
electrode  at  the  place  which  one  wishes  to  be  indifferent  (at  which  if  possible 
no  contractions  are  to  be  made  manifest),  which  possesses  slighter  density 
of  current,  and  by  placing  this  electrode  precisely  upon  indifferent  places 
(sternum,  spinal  column) ,  where  no  nerves  are  located  directly  below  the 
surface  of  the  skin.  This  "loop  of  current"  from  the  indifferent  electrode 
may  really  become  disturbing — if  it  be  a  question  of  determining  precisely 
the  place  of  lesion  of  a  nerve  by  electricity,  and  one  follows  the  trunk  of  the 
nerve  with  the  indifferent  electrode  while  the  active  one  is  placed  upon 
the  muscle,  so  that  under  certain  circumstances  the  two  electrodes  approach 
each  other  closely. 

It  should  be  briefly  mentioned  that  an  electrical  (as  a  rule  only  galvanic) 
test  of  the  sense  nerves  may  be  performed.  The  examination  of  the  eye 
is  made  as  follows :  the  indifferent  large  electrode  is  placed  upon  the  sternum 
or  nape,  the  active,  small  (normal)  electrode,  best  covered  with  a  sponge, 
is  placed  on  the  closed  eyelid,  and  now  the  current  closed,  naturally  with 
weak  current  values.  The  law  of  contracture  remains  as  above,  the  reaction 
of  the  nerves  is  an  intensive  light  (sometimes  also  color)  sensation;  the 
threshold  of  stimulation  is  usually  very  low.  For  the  ear,  it  is  best  to  use 
as  electrode,  a  normal  or  a  button  electrode,  placing  this  upon  the  tragus; 
there  appear  buzzing  or  whistling  auditory  sensations  (the  method  is  much 
used  nowadays  for  otological  tests  in  labyrinthine  vertigo,  so-called  voltaic 
vertigo).  Finally  the  sense  of  taste  may  be  tested  (small  active  button 
electrode  on  the  tongue  or  cheek),  upon  which  the  stronger  sour  taste 
appears  at  the  cathode,  the  weaker  salty  taste  at  the  anode.     This  fact, 


52  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

moreover,  may  be  used  to  ascertain  the  position  of  the  pole,  if  one  does  not 
know  it;  one  places  an  electrode  on  each  cheek,  and  passes  a  weak  current 
through;  the  side  upon  which  a  usually  distinct  sour  sensation  appears, 
corresponds  to  the  positive  pole,  to  the  anode. 

Quantitative  changes  in  electrical  excitability  of  the  muscles  and  the  nerves 
in  unhealthy  conditions  are  alone  shown  when  the  faradic  current  is  used; 
nerves  and  muscles  are  less  or  more  excitable  than  in  the  normal  con- 
dition; with  the  galvanic  current,  on  the  other  hand,  quantitative  and 
qualitative  changes  are  shown.     We  shall  now  take  up  the  former. 

Diminution  and  even  cessation  of  faradic  excitability  is  found  for  the 
muscles  as  well  as  for  the  nerves  in  all  possible  diseases  of  the  peripheral 
neurons.  Its  combination  with  qualitative  changes  in  galvanic  excitability, 
as  in  the  reaction  of  degeneration,  will  be  mentioned  below.  Mere  diminu- 
tion is  found  in  all  simple  (that  is  not  degenerative,  cf.  below)  atrophies,  in 
some  cerebral  and  spinal  paralyses,  in  dystrophy,  occasionally  too,  in  other 
diseases .  that  lead  to  degenerative  atrophy  (neuritis,  poliomyelitis,  etc.), 
and  then,  as  a  rule,  as  the  first  or  last  stage  of  the  electrical  change;  finally  also 
in  the  rare  myotonia  (cf .  above) .  An  increase  in  faradic  excitability  is  found 
almost  exclusively  in  tetany  and  even  there  not  constantly.  Increase  or 
decrease  in  faradic  excitability  is  recognized  simply  by  the  increase  or  de- 
crease of  the  gradations  for  the  minimal  twitching,  as  compared  with  the 
mean  values. 

Increase  in  galvanic  excitability  is  found  (except  in  the  beginning  of  R.  D., 
cf.  below)  also  only  in  tetany,  and  here  constantly  for  the  excitation  of  the 
motor,  frequently  for  that  of  the  sensory  and  sense  nerves  (especially  of  the 
acusticus);  it  is  recognized  by  the  small  power  of  the  current  necessary  to 
produce  the  minimal  twitching.  Decrease  in  galvanic  excitability  is  found 
simultaneously  with  that  of  faradic  excitability  in  the  diseases  mentioned 
above  (at  least  for  the  indirect  stimulation — that  of  the  nerves). 

More  important,  and  in  certain  diseases  far  more  common,  is  the  appear- 
ance of  the  reaction  of  degeneration  (R.D.).  In  this  we  must  distinguish 
clearly  between  the  electric  condition  of  the  nerves  and  that  of  the  muscles. 
In  the  "total"  reaction  of  degeneration: 

(a)  The  nerve  is  excitable  neither  faradically,  nor  galvanically. 

(b)  The  muscle  is  not  excitable  faradically,  on  the  other  hand  its  galvanic 
excitability  is  increased  upon  direct  stimulation  (only  at  the  beginning  of  the 
disease) ;  besides  this  the  law  of  contraction  is  so  changed  for  the  muscle  that 
there  appears  at  first  reaction  (to  the  weakest  stimulation),  a  twitching  upon 
anodal  closing  or  even  upon  its  opening  ("reversal  of  the  law  of  contraction"). 
Besides  this,  the  twitching  becomes  sluggish,  that  is,  the  contraction  rises 
gradually  to  its  height,  and  then  sinks  again  gradually,  slowly;  the  difference 
in  the  twitching  curve  in  direct  stimulation  of  the  muscles  is  about  that  seen 
in  Fig.  17. 


DEGENERATION,  MYOTONIC  AND  MYASTHENIC  53 

There  is  also  a  partial  reaction  of  degeneration,  generally  in  less  severe 
lesions,  or  as  preceding  or  following  a  stage  of  total  lesions;  here  the  only 
f aradic  muscular  excitability  is  decreased  or  even  lost,  and  sluggish  twitching 
is  demonstrable;  on  the  other  hand,  the  excitability  of  the  nerves  may  be  re- 
tained for  both  kinds  of  currents.  The  distinction  of  the  total  from  the  par- 
tial R.  D.  may  be  important  diagnostically  as  well  as  prognostically,  since 
the  latter  trouble  is  cured  considerably  more  quickly. 

The  reaction  of  degeneration  occurs  everywhere,  where  degenerative 
processes  exist  in  the  anterior  horns,  nerves,  or  muscles.     Here  belong 

poliomyelitis  ant.,   syringomyelia,   tumors, 

fl  /        \^  myelitis,  bulbar  paralysis,  spinal  muscular 

/  /  /  N.         atrophy,  amyotrophic  lateral  sclerosis;  all 

Normal  sluggish  contraction.       peripheral   nerve   lesions;    finally   in   rare 

contraction  cases,  some   muscular   diseases,  as  to  the 

Fig.  17. — Diagram  of  the  normal  and  '  ' 

the  sluggish  contraction  in  closing  the    genesis  of  which  we  have  not  yet  full  knowl- 

galvanic  current.  i        /j  i     \ 

edge  (dystrophy). 

We  must  mention,  that,  as  excellent  investigations  of  Grund  have  con- 
firmed, the  influence  of  cold  may  evoke  the  picture  of  partial  reaction  of 
degeneration  (sluggish  twitching,  equalization  and  even  preponderance  of 
the  anode  over  the  cathode)  {"cooling  off  reaction").  The  curves  of  these 
twitchings  are  not  identical  with  those  of  the  R.  D.,  but  even  exceed  them  in 
sluggishness.  This  "cooling  off  reaction"  may  appear  spontaneously  in  the 
small  muscles  of  the  hand  of  the  healthy,  and  persist  for  a  very  long  time 
(for  hours  even  in  a  very  warm  room) ,  evince  itself  also  in  the  larger  muscles 
of  the  body  upon  a  particular  cooling  off,  and  it  may  appear  precisely  in 
diagnostically  important  cases,  in  which,  then,  warming  of  the  muscles  is 
necessary  to  ascertain  whether  a  partial  R.  D.  is  present. 

In  spite  of  this  possibility  of  a  confusion,  the  diagnostic  value  of  R.  D.  is 
very  great;  we  must  emphasize  again  the  special  importance  of  recognizing 
the  sluggish  twitching. 

The  myotonic  reaction  of  Thomsen's  disease  (myotonia  congenita)  pre- 
sents a  peculiarly  abnormal  disturbance  in  electrical  excitability.  Here, 
upon  direct  galvanic  and  faradic  stimulation  of  the  muscles,  there  appears 
even  with  weak  currents,  a  "persistent"  tonic  contraction  of  very  consider- 
able duration;  the  My.  R.  is  especially  striking  upon  weak  galvanic  stimula- 
tion. Stronger  faradic  currents  effect  also,  on  the  part  of  the  nerves,  a  tonic, 
permanent  contraction.  The  phenomenon  is  demonstrable  in  the  (usually) 
hypertrophic,  as  well  as  (in  the  rare  cases  of  this  type)  in  the  atrophic,  muscles 
of  such  patients,  occasionally  very  similar  to  R.  D.,  but  always  without  rever- 
sal of  the  law  of  contraction. 

As  " myasthenic  reaction"  (Mya.R.),  one  designates  an  abnormal  tend- 
ency to  fatigue,  for  the  faradic  current,  on  the  part  of  the  nerves  as  well  as  of 
the  muscles,  appearing  in  myasthenia  gravis  pseudo  paralytica.     If  one 


54 


GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 


repeats  at  short  intervals,  faradic  stimulations  of  equal  strength,  after  some 
(possibly  8  or  10)  stimulations,  a  rapid  decrease,  even  a  disappearance  of 
excitability,  will  be  observed.  In  rest,  the  muscle  and  nerve  soon  "recover" 
again.  Upon  long  lasting  faradization,  the  contraction  decreases  from 
second  to  second  and  finally  disappears  (Jolly). 

My.  R.  and  Mya.  R.  correspond  throughout  to  the  analogous  changes 
in  active  and  mechanical  excitablity  in  myotonia  or  myasthenia.  Some 
rare  electric  changes  will  be  discussed  in  the  separate  chapters. 

D.  Sensibility 

The  disturbances  in  sensibility  in  their  various  forms  may  be  subjective 
and  objective,  but  in  no  process  in  the  human  body  is  there  a  greater  lack  of 
harmony  between  the  subjective  troubles  of  the  patients  and  the  objective 


N.  cutan.  brachii  lateralis  (N. 
axillaris) . 


Nn.  supraclavicularis. 


Rami  cutanei  laterales  Nn. 
intercostalium. 


N.  cutaneus  brachii  medial. 


Rami  cut.   brachii  anterioris  des   N. 
cut.  antibrachii  medialis. 


N.  cut.  brachii  posterior  (N.  radialis). 


N.  cutan.  antibrachii  lateralis 
(N.  musculo-cutaneus) . 


. .    Ram.  volaris  des  N.  cutan.  antibrachii 
medialis. 


Ram.  cut.  palmaris  des  N.  medianus. 
Ram.  superficial,  des  N.  radialis. 


Nn.  digitalis  volaris  des  N.  medianus.    ^--r.-jj-l 


Ram.  cutan.  palmaris  des  N.  ulnaris. 


Ram.  superficialis  des  N.  ulnaris. 


Fig.  18. — Distribution  of  the  cutaneous  nerves  in  the  upper  extremities.     (After  Toldt.) 


findings,  than  in  disturbances  in  sensibility;  nowhere  do  the  deficiencies  in 
our  technique  appear  more  sharply  than  here  (Figs.  18  to  24). 

A  glance  at  the  way  sensibility  is  tested  will  confirm  this.  We  distinguish 
first,  quite  roughly,  the  methods  of  testing  the  separate  qualities  of  sensation 
(the  designation  "  quality, "  however,  has  by  no  means  been  proved  justifiable 


TA  C  TILE  SEXSIBILI T  Y 


55 


in  every  direction) ;  common  to  all,  is  naturally  the  great  defect,  that  in  making 
the  test  we  are  (with  a  few  exceptions)  absolutely  dependent  upon  the 
patient's  statement. 

We  test  first  the  sense  of  touch.  We  have  the  patient  close  his  eyes,  or 
cover  them,  and  touch  in  succession  with  our  finger  tip  the  various  portions 
of  the  surface  of  the  body.  I  consider  this  simplest  of  all  methods  the  best, 
because  it  alone  affords  the  examiner  possibility  of  certain  control  of  the 
pressure  in  the  experiment,  and  alone  permits  keeping  touch  at  the  same 
pressure.     In  the  otherwise  much  used  test  by  means  of  a  small  brush,  the 


X.  supraclavicular  • 


Ram.  cut.  lateralis  N.  intercostalium. 


N.  cut.  brachii  lateralis  (X.  axillaris). 


N.  cutan.  branchii  medialis.  -•  -- 


N.  cut.  brachii  posterior  (X.  radialis). 


Ram.  ulnaris  of  the  X.  cutan.  antibra- 
chii  medialis. 


XT.  cut.  antibrachii  lateralis  (X. 
musculocutaneus) . 


X.  cut.  antibrachii  dorsalis  (X. 
radialis) . 


Ram.  dorsalis  manus.  N.  ulnaris. 


Nn.  digital,  volares  proprii.  of -the  N-  ^_ 
ulnaris. 


Ram.  superficial  of  the  X.  radialis. 


"-"•Si    Xn.  digitatis  volares  proprii  of  the  X. 
medianus. 


Fig.  19. — Distribution  of  the  cutaneous  nerves  in  the  upper  extremities.      {After  Toldt.) 

touch  in  itself  is  always  very  light,  but  a  fine  gradation  of  the  pressure  of  the 
brush  touch  is  almost  an  impossibility,  and  yet  there  occurs  often  the  neces- 
sity for  ascertaining  even  very  fine  distinctions  in  touch  sensibility.  The  test 
of  the  "  touch  circles  "  of  the  skin  by  simultaneously  placing  upon  it  the  points 
of  a  compass  at  varying  distances — which  answers  at  the  same  time  for  a  test 
of  "orientation" — the  patient  to  state  whether  he  feels  one  or  two  points  of 
contact,  when  the  points  of  the  compass  are  at  a  definite  distance  from 
each  other,  may  also  be  dispensed  with,  since  even  upon  the  normal  skin  it 
gives  widely  varying  results. 


56 


GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 


It  is  best  to  carefully  touch  first,  parts  of  the  body  which  have  a  good 
sense  of  touch,  only  then  those  that  have  less  sensibility,  and  let  the  patient 
respond  to  every  touch  he  feels  with  the  word  "now."  But  here  it  is  nec- 
essary occasionally  to  touch  also  distant  parts  of  the  skin,  in  order  to  avoid  a 
sequence  of  symmetrical  points  on  extremities  (which  the  patient  himself  for 
the  most  part  unconsciously  compares)  and  a  definite  rhythm  of  the  touches, 
since  through  all  these  procedures  the  task  of  the  patient  is  made  too  easy,  his 
attention  too  little  occupied,  and  I  recommend  also  asking  from  time  to  time 
"Do  you  feel  this?  this?  this?" — occasionally  asking  the  question  without 
really  touching  the  skin.     Nothing  else  so  sharpens  the  attention.     If  one  has 

Skin  distribution  fifth  nerve 


Skin  distribution 
fifth  nerve 


Skin  distribution 
brachial  plexus 


Lateral  cutane- 
ous intercostal 
branches 


Skin  distribution 
sacral   plexus 


Skin  distribution 
cervical  plexus 


Skin  distribution 
brachial  plexus 


Lateral  cutane- 
ous branches  (in- 
tercostal) 


N.  dorsalis  penis 


Skin  distribution  lumbar  plexus 
Figs.  20  and  21. — Distribution  of  the  cutaneous  nerves  in  the  trunk.     {After  Toldt.) 

found  parts  with  lessened  sensibility  to  touch,  one  should  try  to  make  a 
boundary  between  these  and  those  that  have  the  normal  sensibility  and  im- 
mediately observe  whether  this  boundary  is  of  a  segmentary  nature,  follow- 
ing the  distribution  of  the  individual  cutaneous  nerves,  or  of  a  hysteric  (glove- 
and  sleeve-like,  unilateral,  etc.)  character.  One  should  note  also  whether 
the  touch  which  is  felt  everywhere  may  not  be  in  some  regions  (affected  with 
paresthesia)  felt  "different"  than  in  those  with  normal  sensations,  which  is 
not  rarely  the  case.  For  these  cases  and  for  the  lightest  objective  disturb- 
ances in  sensibility,  it  is  also  recommended  to  let  the  patient  decide  whether 
he  is  being  touched  with  the  "point"  or  the  "eye"  (sharply  or  bluntly)  of 
a  needle  which  is  not  too  sharp;  with  some  practice,  the  disturbing  factors 
of  pain  and  pressure  that  are  easily  produced  here  may  be  avoided. 

The  mucous  membranes  which,  on  the  whole,  show  somewhat  slighter 
sensibility  than  the  skin,  may  be  tested  in  like  manner,  but  the  results  are  then 
less  reliable  (cf.  below). 


THERMAL  SENSIBILITY 


57 


The  normal  sensibility  of  the  skin  varies  in  delicacy  in  the  different  parts 
of  the  body,  being  finest  at  the  ends  of  the  fingers  and  the  tip  of  the  tongue, 
and  dullest  (even  to  anaesthesia)  upon  the  callous  portions  of  the  soles  of  the 
feet. 

If  we  have  examined  the  touch  sensibility  and  noted  the  disturbance  that 
we  may  have  found  upon  a  diagram  of  the  body  (which  is  earnestly  recom- 
mended), we  proceed  to  test  the  temperature  sense  or  more  correctly  the  sensa- 
tions of  heat  and  cold.    For  this  we  use  test  tubes,  filled  with  warm  and 


Ram.  cutaneus  lateralis  des  N. 
iliohypogastricus. 


N.  cutan.  femoris  lateralis. 


N.  lumboinguinalis. 


N.  ilioinguinal.     N.  spermaticus 
extemus. 


Ram.  cutan.  femoris  arteriores. 


Ram.  cutan.  N.  obturatorii. 


N.  cutan.  surae  lateralis  {a,  d,  N. 
peronaeus). 


\J/ 


Ram.   cutanei  cruris   mediales  des  N. 
saphenus. 


N.  tibialis. 


N.  peron.  superficial. 


~-~"   N.  peron.  profundus. 
Fig.  22.— Distribution  of  the  cutaneous  nerves  in  the  lower  extremities.     {After  Toldt.) 


with  cold  water  (no  extreme  degrees  of  heat  and  cold,  through  which  pain 
sensations  are  evoked),  possibly  also  for  a  quick  test  the  warm  hand  and  the 
cold  metal  of  the  percussion  hammer,  and  proceed  in  the  same  manner  as 
described  above.  As  a  rule,  the  disturbances  thus  established  will  coincide 
more  or  less  with  those  of  the  touch  sense,  though  not  exactly  at  the  bound- 
aries. This  is  partly  due  to  the  fact  that,  as  Goldscheider  has  recently 
established  on  the  basis  of  careful  investigations,  the  skin  has  special  points 
of  pressure,  cold  and  warmth,  that  is,  places  (and  especially  nerve  ends), 
where  touch,  cold  or  warmth,  may  be  best  felt.  But  as  Oppenkeim  pointedly 
remarks,  it  would  be  very  difficult  clinically  to  proceed  to  investigate  the 


58 


GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 


surface  of  the  skin,  as  laid  out  by  Goldscheider  according  to  this  scheme,  as  to 
areas  possessing  varying  degrees  of  sensibility,  especially  as  this  procedure 
scarcely  ever  gives  more  important  results  than  are  obtained  by  the  usual  test. 
In  another  respect  also,  I  recommend  a  practical  deviation  from  Gold- 
scheider''s  proposals,  namely,  in  the  test  of  pain  sensations.  Granted,  that 
special  points  of  pain  and,  therefore,  pain-nerves  too,  can  not  be  found  in  the 
skin,  and  that,  on  the  other  hand,  by  increasing  almost  every  stimulation  of 
any  sensory  quality,  the  sensation  of  pain  may  be  evoked,  still,  clinically,  we 
can  not  dispense  with  the  concept  of  pain  sensation — first  because  of  the 
patient's  psyche,  usually  very  susceptible  to  the  thought  of  pain  (though, 


N.  clunium  medii.    ~T~ 


•  Nn.  clunium  superiores. 


--»  Nn.  clunium  inferiores. 


Cutan.  branches  femor.  anteriores. 


.__  N.   cutaneus    femor.    lateralis    (Plex. 
lumbal). 


Cutaneus  branches  N.  obturatorii. 


1  N.  cutaneus  femor.  posterior. 


Cutan.  branches  cruris  mediales. 


Branches  calcan.  mediale  (N. 
tibialis) . 
Branches  calcan.  lateralis  (N.  suralis) . 


N.  cutan.  suras  lateralis  (from  N. 
peronaeus). 


N.  tibialis. 


N.  peronaeus  superficialis. 


N.  plantaris  lateralis. 
-—  (N.  tibialis). 


N.  plantar  mebialis  (N.  tidialis).  —-"■"" 
Fig.  23. — Distribution  of  the  cutaneous  nerves  in  the  lower'extremities.      (After  Toldt.) 

doubtless  loss  of  the  pain  sensation  is  often  observed  only  late),  secondly, 
because  frequently  in  sensibility  disturbances  of  functional  and  even  of 
organic  nature,  only  these  more  intensive,  pain-producing  stimulations  show 
functional  changes.  It  is  best  to  test  the  pain  sensation  simply  with  a  sharp 
needle  and  by  pinching  folds  of  raised  skin,  or,  further,  by  the  electric 
current,  making  use  of  fine  electrodes.  The  needle  point  is  also  the  principle 
made  use  of  in  the  numerously  used  apparatus  for  measuring  pain  (alges- 
imeters) ,  in  which  a  sharp  needle  may  be  screwed  out  for  a  greater  or  lesser 
distance  from  a  flat  surface  upon  the  instrument;  when  the  needle  protrudes 
only  slightly,  so  as  not  to  pierce  the  epidermis,  the  application  of  the  instru- 


PAIN  SENSE,  SENSE  OF  LOCALITY  59 

merit  is  felt  only  as  a  touch,  gradually,  with  the  lengthening  of  the  needle,  as 
pain.     All  algesimeters  may  be  regarded  as  useless  for  clinical  service. 

Whether  the  psycho -galvanic  reflex  phenomenon  described  by  Veraguth 
(a  galvanometer  inserted  into  the  circuit  of  bodily  conduction  shows  deflec- 
tions in  psychic  processes,  therefore  also  in  pains,  no  beat  is  visible  in  the 
stimulation  of  analgesic  zones)  will  be  able  to  fill  the  gap,  seems  for  the 
present,  very  doubtful;  but,  not  having  peisonal  experience,  I  shall,  for  the 
time  being,  refrain  from  pronouncing  judgment.  For  certain  zones  of  the 
body  there  are  moreover  useful  reflex  phenomena  for  judging  the  pain 
sensation,  as,  for  instance,  dilatation  of  the  pupils  which  occurs  after  painful 
stimulation  of  the  skin  of  the  cheek,  which  is  non-existent  when  the  cheek  is 
analgesic. 

The  sensitiveness  of  the  skin  to  pain  is  not  constant  over  the  entire  body, 
being  greatest  in  the  skin  of  the  forehead,  least  in  the  region  of  the  buttocks. 
At  every  test  of  the  pain  sense,  one  must  watch,  besides  the  intensity  of  the 
sensation,  also  the  period  of  latency  between  the  touch  and  the  sensation, 
since  in  no  other  sensory  quality  does  such  considerable  "retardation" 
occur  (cf.  below).  The  test  may,  in  general  (except  particular  cases,  cf. 
below),  in  order  to  spare  the  patient,  be  confined  to  those  parts  of  the  body 
in  which  one  of  the  previous  examinations  demonstrated  the  presence  of 
a  disturbance. 

The  test  of  the  sense  of  locality,  that  is,  the  keenness  in  the  recognition 
of  the  places  upon  the  skin  that  are  touched,  may  without  trouble  be  com- 
bined with  the  testing  of  the  sense  of  touch,  by  having  the  patient,  in  the 
more  delicate  tests,  say  where  he  was  touched.  As  a  rule,  the  results  do 
not  differ  essentially  from  those  obtained  by  testing  the  sense  of  touch. 
(The  test  by  means  of  the  compass  is  in  a  strict  sense — cf.  above — also  a  test 
of  the  localization  sense.)  To  the  examinations  of  the  actual  sensibility 
of  the  skin  there  belongs,  furthermore,  according  to  some  authors,  the  hair 
sensibility,  that  is,  the  sensitiveness  to  a  slight  touch  upon  the  hair  alone,  not 
upon  the  skin  beneath  it.  The  areas  of  most  delicate  hair  sensation  are  by  no 
means  identical  with  those  of  the  most  delicate  skin  sensation  (but  the  sup- 
position that  the  skin  underlying  the  hairy  parts  of  the  body  is  always  less 
sensitive,  is  not  justifiable).  The  method  has  not  led  as  yet  to  practically 
important  results.  The  examination  of  far  ado-cutaneous  sensibility,  occasion- 
ally used  in  testing  the  sense  of  touch,  that  is,  the  feeling  of  tickling  (formi- 
cation) to  weak  faradic  currents,  seems  to  give  results  identical  with  those  of 
touch  and  pain  sensation,  so  that  this  finely  graded  and  measurable  method 
(determination  of  the  stimulation  threshold  of  sensation  by  means  of  the 
gradation  of  the  current)  offers  no  special  advantages.  The  assertion  that 
certain  poisons  (bisulphide  of  carbon)  mainly  disturb  farado-cutaneous 
sensibility,  has  not  been  established. 

Far  more  important  are  investigations  of  the  deep  sensibility  (bones, 


60  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

joints,  muscles).  According  to  Goldscheider ,  instead  of  the  expression 
formerly  used  " muscular  sense"  which  comprises  the  main  mass  of  deep 
sensibility,  one  should  use  its  separate  components:  the  sensation  of  active 
and  passive  movements,  the  sensation  of  weight  and  resistance  (also  called 
strength  sensation)  and  perception  of  situation.  Stereo  gnosis  (recognition  of 
the  form  and  other  external  qualities  of  objects  by  means  of  touch)  again 
embraces  several  of  these  qualities  in  another  grouping,  as  well  as  the  most 
delicate  psychic  processes  (mnemic  images) .  As  an  independent  sensation, 
bone  sensibility  would  have  to  be  added  to  this.  All  these  forms  of  sensibility 
may  be  disturbed  individually,  and  in  groups,  with  and  without  participation 
of  the  skin  sensation. 

The  sensation  of  active  movements  may  be  tested  in  the  patient  only 
with  great  difficulty,  if  it  occurs  in  isolation.  When  he  is  told  to  perform 
certain  movements  and  then  to  state  the  effect  of  movement,  the  situation 
into  which  the  limbs  were  brought,  the  sensation  of  position  is  virtually  tested 
(cf .  below) .  Unquestionably,  there  exist  in  a  sound  person  sensations  of  active 
movement,  the  loss  of  which  is  felt  as  a  disturbance  (cf .  below) ;  but  an  actual 
test  can  scarcely  be  performed.  The  test  of  the  sensation  for  passive  move- 
ments is,  on  the  other  hand,  very  easy.  With  one  hand,  the  examiner  fixes 
one  of  the  patient's  extremities,  and  with  the  other,  he  performs  as  slight 
excursions  as  are  possible  in  small  joints  in  the  peripheral  parts  of  the  ex- 
tremities, small  movements  of  the  toes,  excursions  of  the  fingers,  etc.  The 
patient,  who  naturally  must  have  his  eyes  closed  or  bandaged,  states  the 
direction  of  the  movement.  Tests  with  considerable  excursion  in  the  joints 
concerned  should  not  be  made,  because  through  tension  of  the  skin,  tendons, 
etc.,  the  patient  is  supplied  with  other  possible  sources  of  knowledge.  For 
the  same  reason,  the  test  for  the  large  joints  (knee,  hip)  can  not  be  used 
extensively  because  of  the  large  areas  of  skin  concerned.  The  sensation  of 
weight  and  resistance,  the  weight  sense,  may  be  tested  in  intelligent  patients, 
by  allowing  them  to  raise,  with  their  eyes  closed,  loads  of  various  weight  (with 
the  entire  extremity,  or  with  individual  parts  of  it,  forearm,  finger,  etc.),  and 
to  tell  the  difference  in  weight.  As  a  rule,  this  sensation  is  disturbed  only  in 
combination  with  others;  moreover,  the  poor  valuation  ability  of  most 
patients  destroys  its  usefulness,  which  otherwise,  for  instance,  might  easily 
be  shaded  more  minutely  by  the  dynamometer,  upon  which  one  lets  the 
patient  exert  certain  predetermined  degrees  of  pressure. 

The  feeling  of  posture,  on  the  other  hand,  permits  of  more  exact  ex- 
amination. One  places  the  extremity  (or  part  of  it)  in  a  position  contrary 
to  that  of  the  natural  posture  in  rest,  and  has  the  patient  state  what  the  new 
position  is  like,  about  how  far  it  is  removed  from  the  position  of  rest,  etc. 

The  stereo  gnostic  sensation  may  also  be  very  easily  tested;  since  this  is 
but  very  slightly  developed  without  actual  touch,  it  is  best  to  put  the  object 
to  be  tested  directly  in  the  hand  of  the  patient,  and  to  let  him  describe  its 


STEROGNOSIS,     BONE  SENSIBILITY  61 

form,  size  and  substance.  For  this  we  choose  as  varied  objects  as  possible, 
which  are,  however,  known  by  their  form  (coins,  keys,  dice,  balls,  fabrics 
like  velvet,  silk,  etc.).  As  has  been  observed  above,  several  kinds  of  sen- 
sation are  involved  in  this  recognition. 

Dejerine  and  Egger  designated  as  bone  sensation,  the  sensation  of  the 
vibration  produced  by  a  vibrating  tuning  fork  when  placed  upon  a  part  of 
the  body  where  the  bone  is  covered  only  by  the  skin.  They  consider  it  an 
independent  sensation,  because  it  may  be  disturbed  without  the  tactile 
disturbance  of  the  skin,  and  vice  versa.  This  fact  is  probably  quite  correct, 
as  German  authors  were  able  to  prove  also  in  the  subsequent  test  (partly, 
however,  it  is  designated  pallaesthesia,  and  ascribed  not  only  to  the  bones 
but  also  to  all  other  subcutaneous  tissue  layers);  but  since  this  vibration 
feeling  is  especially  common  with  simultaneous  disturbances  of  the  "  muscu- 
lar sense"  (tabes),  a  particularly  far  reaching  result  could  scarcely  be 
expected,  and  has  not  yet  been  attained. 

Our  knowledge  as  to  the  sensibility  of  the  internal  organs  in  the  narrower 
sense,  is  by  no  means  good.  The  mucous  membranes  of  the  cavities  com- 
municating with  the  air  (mouth,  nose,  cornea)  show  the  same,  though  in 
general  somewhat  lowered  sensibility,  as  that  of  the  external  skin,  as  we 
have  mentioned  before.  This  is  not  true  of  the  pleural  and  peritoneal 
cavities,  the  lungs,  stomach  and  intestines,  the  bladder  and  kidneys.  Among 
all  these  formations,  the  pleura,  peritoneum  and  the  mucous  membrane 
of  the  bladder  are  those  only  that  are  certainly  sensitive  to  pain  and  touch. 
The  meninges,  substance  of  the  brain,  lungs,  stomach,  intestines,  on  the 
other  hand,  are  not  sensitive  to  touch,  but  may  very  easily  be  the  seat  of 
spontaneous  pain  sensation,  which  is  probably  induced  by  the  sympathetic 
(L.  R.  Mutter).  But  for  a  test  of  these  functions,  we  have  as  yet  no  sufficient 
experience. 

Disturbances  in  sensibility  are  divided  first  into  subjective — paresthe- 
sias and  pains — and  objective;  namely,  hyperesthesia,  hypaesthesia,  and 
anaesthesia. 

Paraesthesia  signifies  the  abnormal  sensations  of  the  various  qualities 
which  are  present  only  subjectively.  They  belong  most  frequently  to  the 
realm  of  the  touch  sensation,  more  rarely  to  the  temperature  sensations; 
the  abnormal  subjective  sensations  from  the  group  of  the  "muscular  sense" 
(cf.  above)  are  rare;  but  frequently  a  tabetic  may  feel  disturbances  in  the 
sensations  of  position,  movement,  etc.,  that  can  not  be  objectively  demon- 
strated (he  does  not  know  how  he  holds  the  foot  in  walking,  etc.;  but  here, 
as  a  rule,  paraesthesia  and  objectively  demonstrable  disturbances  coincide), 
also  in  psycho-neuroses,  there  occur  occasionally  disturbances  of  feeling 
of  posture,  of  stereognostic  recognition,  etc.,  in  purely  subjective  form. 
The  most  common  paraesthesia  is  tickling  (feeling  of  deadness,  of  numbness, 
formication,  etc.),  which  very  frequently  represents  an  early  symptom  of 


62  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

severe  organic  diseases,  and  moreover  is  often  in  so  far  objectively  demon- 
strable that  the  patient  feels  exactly  a  touch  in  the  paresthetic  area,  but 
"differently"  than  in  the  sound  parts  of  the  body.  Disturbances  in  heat 
sensation  are  usually  described  in  very  characteristic  manner:  "I  feel  as  if 
my  legs  were  packed  in  ice"  (myelitis).  A  common  paresthesia,  which 
is  often  combined  with  pains,  is  the  "girdle  feeling";  as  if  a  hoop  were 
placed  around  the  body,  a  cord  about  the  knee  (usually  corresponding  to  an 
affection  of  the  posterior  roots).  In  many  paresthesias,  their  purely 
psychogenous  origin  may  be  recognized  at  once  (for  instance  a  feeling  of  a 
living  animal  in  the  stomach,  globus).1 

Pains  represent  the  most  difficult  subject  matter  of  neurologic  (often, 
too,  of  internal)  diagnostics.  Only  in  extremely  rare  cases  can  the  spon- 
taneous pain  be  recognized  by  the  examiner  (occasionally  through  vaso 
motor  symptoms,  reddening  of  the  face,  pallor,  fluctuations  in  blood  pressure, 
local  coolness  or  warmth) — most  often  in  pains  evoked  through  the  sympa- 
thetic paths  (vascular  crises,  etc.),  in  which  we  may  find  conditions  of 
collapse,  superinduced  by  the  pain,  which,  however,  occur  also  in  purely 
"sensory"  pains.  Unfortunately,  we  have  no  means  for  measuring  the 
spontaneous  pain  (cf.  above).  In  every  case,  we  let  the  patient  describe 
his  pains  as  accurately  as  possible;  their  localization  (following  nerve 
trunks,  diffuse,  corresponding  to  an  internal  organ — cf.  below — confined 
to  one  vascular  realm),  their  character  ("nagging,"  "boring,"  "pressing," 
the  lightning-like  "lancinating  pains,"  "colic-like"  pains);  their  duration 
may  often  be  of  great  significance. 

Of  the  objective  disturbances  in  sensibility,  the  hyperesthesias  are  com- 
paratively the  rarest.  Tactile  hyperesthesia,  the  feeling  of  an  unpleasant 
sensation  of  pain  upon  the  slightest  touch,  is  found  occasionally  in  neu- 
ralgias, but  also  in  pure  neuroses  and  in  diseases  of  the  internal  organs  in 
the  so-called  "zones  of  Head"  (cf.  below).  Hyperesthesia  to  cold  and 
(far  more  rarely)  to  heat  is,  though  not  a  common,  still  a  characteristic 
symptom  of  organic  diseases  (tabes).  Hyperesthesia  to  pain  or  hyper- 
algesia, that  is,  feeling  of  a  violent  pain  upon  a  touch  that  usually  causes 
but  a  disagreeable  sensation  or  a  slight  pain,  is,  on  the  other  hand,  found 
rather  frequently  in  all  sorts  of  organic  (pressure  points  in  neuralgias  of 
this  kind)  and  psychogenous  (pvaralgia  in  hysteria)  diseases. 

The  group  of  hypcesthesias  or  anaesthesias,  the  partial  or  total  loss  of 
the  various  qualities  of  sensation  (the  single  forms  are  known  also  as  thermo- 
anesthesia, hypalgesia,  asterognosia,  pallanesthesia),  is  the  largest.  This 
is  the  point  in  which  the  test  of  the  separate  sensory  qualities  has  the  most 
valuable  results.  We  must  first,  as  has  been  stated  above,  consider  the 
distribution  of  the  zones  of  such  sensory  disturbance  (Fig.  24).     Here  we 

1  On  the  other  hand,  the  parses thesias  of  the  sense  nerves — entoptic  and  entotic  phenomena — 
are  also  frequently  found  in  non-psychogenous  diseases. 


TYPES  OF  AXMSTHESIA 


63 


find  four  main  forms  of  distribution  of  these  disturbances:  types  of  total 
break  in  conduction  in  the  central  organ  (sensory  hemiplegia,  paraplegia — 
occurring  from  organic,  as  well  as  from  psychic  causes) ;  disturbances  in  the 
area  subserving  the  individual  segments  and  roots  of  the  spinal  cord  (cf .  the 
segment  schemata  in  the  special  pathology  of  the  spinal  cord) ;  disturbances 
in  the  distribution  area  of  the  peripheral  sensory  nerves,  for  which  the  sche- 
mata subjoined  will  serve  as  points  of  reference;  finally  disturbances  of  psy- 
chogenous  kinds  ("central  type"),  which  are  restricted  to  none  of  the  forms 
described   (glove,    sleeve-stocking-like    anaesthesias,    usually    in    hysteria). 


Fig.  24. — The  three  types  of  disturbances  in  sensibility  at  the  extensor  side  of  the  right  upper 
extremity.  (After  Obersteiner  and  Redlich.)  A.  Peripheral  type.  1,  X.  supraclavicularis-  2  N. 
axillaris;  3,  X.  cutan.  post.  sup.  radialis;  4,  lateral  branches  n.  spinal;  5,  X.  cutan.  medial  radialis; 
6,  X.  cutan.  post.  inf.  radialis;  7,  X.  cutan.  med.;  8,  X.  cutan.  lateralis;  9,  X.  radialis;  10,  X. 
ulnaris;  n,  X.  medianus.  (After  Bernhardt.)  B.  Segmental  type,  4,  5,  6,  7,  8.  4-8,  Cervical 
segment;  I,  1.  Dorsal  segment.     (After  Allen  Starr .)     C.  Central  type. 

To  which  of  the  groups  mentioned,  the  hypaesthesia  or  anaesthesia  under 
consideration  belongs — a  question  often  decisive  for  diagnosis — is  a  matter 
that  can  be  settled  only  by  determining  accurately  the  boundaries  of  the 
disturbance.  Inasmuch  as  an  anaesthetic  area  but  rarely  touches  directly 
one  that  has  normal  sensibility,  this  determination  demands  much  patience 
and  skill. 

We  must  also  mention  here,  that  Head  succeeded  in  finding  for  diseases 
of  the  internal  organs  (lungs,  stomach,  intestines,  etc.),  cutaneous  nerve 
areas,  which  synchronously  display  abnormal  forms  of  sensibility,  according 


64  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

to  what  has  been  determined  so  far,  almost  exclusively  hyperesthesias 
("reflex  hyperesthesias "  best  known  in  gastric  affections,  as  band-like  or 
singularly  bounded  zones  of  half  the  abdomen,  of  the  back,  etc.).  We  can 
not  here  enter  upon  a  detailed  discussion  of  this  subject. 

As  has  already  been  said,  each  quality  of  sensibility  may  be  individually 
disordered,  or  they  may  all  be  affected  at  the  same  time.  We  find,  rather 
frequently,  also,  an  affection  by  groups;  only  the  cutaneous,  or  only  the  deep 
sensibility.  The  most  common  of  these  forms  of  a  partial  or  "dissociated 
sensibility  disturbance''''  is  probably  that  of  simultaneous  disturbance  of  the 
pain  sensation  and  of  the  cold  and  heat  sensation,  while  the  tactile  sensibility 
remains  normal,  as  in  syringomyelia. 

Some  rather  rare  disturbances  shall  be  briefly  mentioned.  The  pain 
sensation  is  not  rarely  disturbed  in  such  a  way,  that  the  pain  stimulation  is 
felt  only  late,  after  a  period  of  latency  of  one  or  more  seconds,  that  is,  is  felt 
as  pain;  as  a  rule,  the  touch  is  at  once  registered  as  such,  and  only  the  pain 
appears  later  (delay  or  retardation  of  the  pain  sensation;  double  sensation) . 
In  other  cases,  a  single,  brief,  though  intense  pain  stimulation  (prick) 
produces  no  pain;  on  the  other  hand,  pain  is  felt,  if  one  allows  it  to  persist 
for  some  time  with  the  same  degree  of  severity  {summation).  Disturbances 
in  the  temperature  sense  are  most  frequently  the  foundation  of  "perverse 
sensations"  which  are  as  such,  rare;  warm  is  felt  as  cold,  cold  as  touch  or 
pain,  etc.  Allocheiria,  the  localization  of  touch  upon  the  corresponding 
place  in  the  extremity  not  touched,  is,  at  any  rate,  as  rare  an  occurrence  as 
polyasthesia,  in  which  a  single  touch  is  felt  as  if  multiple. 

The  characteristic  disturbance  of  deep  sensibility,  frequently  met  in  central 
organic  nervous  diseases,  affects  most  frequently  the  sense  of  position  and  the 
stereognostic  sensation.  The  patient  in  bed  "no  longer  knows  where  his 
legs  are,  whether  he  has  any  or  not";  he  no  longer  recognizes  objects  placed 
in  his  hands.  Disturbances  in  the  sensation  of  vibration  are  decidedly  less 
common. 

E.  Reflexes 

We  distinguish  tendon  reflexes,  cutaneous  (and  mucous  membrane) 
reflexes  and  internal  reflexes. 

Without  entering  with  more  detail  into  the  concept  of  "reflex"  we  shall 
mention  here  only  that  we  shall  designate  as  "reflex"  in  the  sense  of  the 
phenomena  now  under  consideration,  only  muscular  twitchings,  that' is, 
contractions,  which  follow  a  sensory  stimulation,  with  the  exception  of  the 
idiomuscular  contractions  (cf.  above).  All  these  reflexes,  then,  have  a 
local  motor  effect.  We  regard  in  general,  also,  the  tendon  phenomena 
discovered  by  Erb  and  Westphal,  as  genuine  reflexes,  not  (as  Westphal  held) 
as  direct  stimulation  of  the  muscle  concerned. 


PERIOSTEAL  AND  TENDON  REFLEXES  65 

There  are  an  immense  number  of  tendon  reflexes  in  the  human  body, 
many  of  which  can  be  proved  present,  even  in  the  normally  healthy  individ- 
ual, though  not  all  constantly,  and  some  only  upon  increase  of  the  reflex. 
We  shall  mention  here  only  the  most  important,  the  knowledge  of  which 
quite  suffices  for  the  recognition  of  all  nervous  diseases  involved. 

In  the  head  we  know  only  the  lower  jaw  or  masseter  reflex.  It  is  most 
easily  evoked  as  follows :  When  the  mouth  of  the  patient  has  been  slightly 
opened  the  examiner  places  a  finger  upon  his  lower  jaw  and  with  the  percus- 
sion hammer,  gives  the  finger  a  light  blow  from  above;  there  results  a  raising 
of  the  lower  jaw,  effected  mainly  by  the  masseters,  the  mouth  closing.  The 
same  reflex  may  be  evoked  by  the  periosteum  of  the  lower  jaw  near  the  point 
of  insertion  of  the  masseters;  this  reflex  is  virtually  one  of  the  so-called  perios- 
teal reflexes,  which,  however,  genetically  and  nosologically,  may  be  counted 
among  the  tendon  reflexes.  The  lower  jaw  reflex  is  comparatively  constant 
in  the  healthy. 

The  somewhat  less  constant  forearm  reflexes  are  also  genuine  periosteal 
reflexes  and  may  be  evoked  by  tapping  the  lower  end  of  the  radius  (radial 
periosteal  reflex)  and  the  ulna  (ulnar  periosteal  reflex)  which  causes,  in  the 
(more  common)  radial  reflex,  a  reflex  contraction  of  the  supinator,  that  is,  a 
flexion  of  the  forearm,  in  the  ulnar  reflex,  usually  a  slight  retardation. 
Both  reflexes  are  tested  with  the  forearm  flexed  into  a  right  angle,  ad- 
ducted  and  slightly  pronated,  in  order  to  relax  the  m.  supinator  as  far  as 
possible. 

The  triceps  reflex  is  scarcely  ever  lacking  in  the  healthy,  but  is  often  diffi- 
cult to  test.  The  arm  of  the  patient  is  held  somewhat  in  the  same  flexed 
position  as  mentioned  before,  and  a  short,  quick  blow  with  the  percussion 
hammer,  delivered  upon  the  tendon  of  the  m.  triceps,  right  above  the  ole- 
cranon. There  results  a  movement  of  extension,  often  only  slight,  in  the 
forearm.  The  danger  consists  in  the  possibility  of  striking  the  muscle  itself 
instead  of  the  tendon,  thus  producing  direct  muscular  stimulation,  which,  as 
a  rule,  has  a  slighter  motor  effect,  but  may  otherwise  closely  resemble  the 
reflex.  Since  the  triceps  reflex — a  genuine  tendon  reflex — may  be  of  diag- 
nostic importance  (especially  its  absence  in  tabes,  etc.),  this  point  must  not 
be  overlooked. 

The  tendon  reflexes  in  the  trunk  are  of  no  great  importance.  We  must 
mention  the  scapulo-humeral  reflex  described  by  Bechterew,  a  periosteal  reflex 
produced  by  tapping  the  scapula,  which,  however,  I  have  not  found  suffi- 
ciently constant  to  attach  great  weight  to  its  absence. 

The  most  important  tendon  reflexes  belong  to  the  lower  extremities. 
Important  above  all  is  the  patellar  (kneecap)  reflex,  the  contraction  of  the. 
m.  quadriceps  upon  tapping  the  patellar  tendon  below  the  patella.  It  is  the 
most  constant  of  all  reflexes  in  the  healthy  (lacking  only  in  0.04  %  of  the 
cases)  and  can  be  tested  with  comparative  ease.  For  its  test,  still  more  than 
5 


66  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

for  that  of  all  other  reflexes  the  principle  that  to  evoke  the  reflex  the  muscle 
concerned — here  the  quadriceps — must  be  totally  relaxed,  holds  good. 

Passive  relaxation  is  usually  attained  by  a  certain  position  of  the  leg;  but 
often  even  in  intelligent,  especially  in  timorous,  patients  an  active  relaxation 
is  accomplished  with  the  greatest  difficulty.  We  recommend  the  following 
ways  and  means : 

i.  The  patient  sits  on  a  chair,  crosses  the  leg  to  be  tested  over  the  other; 
the  examiner  delivers  a  horizontal  blow  with  the  inner  edge  of  his  hand  or 
with  the  percussion  hammer  upon  the  quadriceps  tendon  below  the  patella, 
where  it  bridges  over  the  space  between  the  patella  and  tuberositas  tibiae  and 
lies  therefore  in  a  more  or  less  hollow  space  (this  "hollow  space"  is  probably 
the  reason  for  its  remarkable  constancy) .  This  method  is  not  safe  because 
total  relaxation  is  rarely  attained ;  it  has  the  advantage  of  the  largest  motor 
effect  (of  the  sudden  lifting  of  the  tip  of  the  foot)  which,  however,  is  of  com- 
paratively less  importance  because  in  excitation  of  the  patellar  reflex  one 
should  always  pay  more  attention  to  the  contracting  muscle  than  to  the  motor 
effect;  in  decreased  reflexes,  it  often  happens  that  the  former  only  is  visible. 

2.  The  patient  sits  on  a  chair  which  is  to  be  several  centimeters  lower 
than  the  leg  proper  of  the  patient  and  places  the  foot  of  the  leg  to  be 
tested  so  far  forward  that  leg  and  thigh  form  an  angle  of  about  ioo  de- 
grees, and  the  thigh  rests  easily  upon  the  chair.  A  great  many  patients 
are  best  able  to  relax  the  muscle  when  in  this  position;  the  motor  effect  is 
slight,  but  the  contraction  of  the  muscle  is  usually  very  distinct.  I  recom- 
mend this  method,  as  well  as  the  following : 

3.  The  patient  lies  in  bed,  as  flat  as  possible,  with  the  upper  part  of  his 
body  but  slightly  raised;  the  leg  is  somewhat  flexed  so  that  leg  and  thigh  form 
an  angle  of  about  140  degrees  and  at  the  same  time  the  thigh  is  abducted 
(the  knee  of  the  flexed  leg  inclines  to  the  outside).  The  blow  on  the 
patellar  tendon  usually  evokes  an  evident  extension  of  the  leg  proper.  The 
relaxation  of  the  quadriceps  usually  succeeds,  though  not  always  easily. 
Crossing  of  the  legs  in  a  recumbent  position  is  not  so  good. 

4.  The  leg  proper  of  the  recumbent  patient  is  raised  by  means  of  a 
handkerchief  or  some  similar  device  placed  beneath  it,  so  that  the  leg  lies 
horizontally  in  its  support  and  the  thigh  is  flexed  into  obtuse  angles  with  it 
and  the  trunk.  Here,  it  is  supposed,  the  relaxation  is  very  nearly  complete. 
Almost  every  year,  however,  new  modifications  are  recommended  for  testing 
this  reflex,  that  is,  for  relaxing  the  muscle. 

I  should  call  the  Achilles  tendon  reflex,  considering  its  work  and  signifi- 
cance, the  second  of  the  most  important  tendon  reflexes  of  the  body.  It  con- 
sists in  a  contraction  of  the  calf-muscles  from  which  the  tendon  of  Achilles 
courses,  of  the  triceps  suras,  and  thereby  evokes  plantar  flexion  of  the  foot. 
It  is  elicited  by  striking  the  tendon  of  Achilles  with  the  percussion  hammer; 
here,  too,  the  relaxation  of  the  calf  is  the  main  thing.     For  this,  the  position 


DISORDERS  IN  TENDON  REFLEXES  67 

described  above  under  3  usually  suffices;  the  examiner  then  stands  at  the  foot 
of  the  bed  and  from  the  inner  side  of  the  leg  delivers  a  brief  blow  upon  the 
tendon.  Under  certain  circumstances  one  must  test  the  tendon  at  several 
places.  The  absence  of  this  reflex,  as  that  of  the  patellar  reflex  is,  in  every 
case,  pathological  (unless  contractures,  etc.,  prevent  their  appearance). 
Other  methods  for  testing  it  are  the  striking  of  the  tendon  while  the  patient 
kneels  on  a  chair,  or  by  striking  the  tendon  while  the  patient  lies  on  his 
stomach  with  his  leg  flexed  at  the  knee. 

All  other  tendon  reflexes  are  inconstant,  that  is,  demonstrable  only  in 
pathological  increase  of  the  reflex,  even  the  dorsal  foot  reflex  described  by 
Bechterew  and  Mendel  (which  with  Spier  I  would  count  as  a  genuine  tendon 
phenomenon.) 

We  know  two  kinds  of  pathological  changes  in  the  tendon  reflexes: 
increase  and  decrease,  or  absence.  The  former  condition  is  accompanied  by 
increase  of  the  muscular  tonus  (cf.  above),  being  its  most  regular  concomitant 
phenomenon,  the  latter  with  decrease  of  the  tonus  (but  here  the  connection  is 
less  constant). 

We  can  say  with  certainty  when  a  reflex  is  to  be  called  increased  only  in 
the  case  of  the  patellar  and  the  Achilles  tendon  reflexes.  Increase  in  the  reflex, 
as  a  rule,  effects  a  more  extensive  muscular  contraction  with  considerable 
motor  effect  and  also  the  appearance  of  tendon  reflexes  that  are  not  demon- 
strable at  other  times.  In  spastic  conditions  of  high  degree  (i.  e.,  naturally 
only  so  long  as  the  spastic  musculature  is  not  so  tightly  contracted  as  to  make 
further  contraction  impossible)  by  striking  almost  any  tendon  in  the  body 
that  is  to  some  extent  exposed,  one  may  produce  twitchings  in  the  muscle 
concerned;  among  the  most  common  in  this  case  are  twitchings  in  the  adduc- 
tor tendons  of  the  thigh  (adductor  reflex)  and  in  the  tibialis  posticus. 

Increase  in  the  patellar  reflex  is  present  if  the  reflex  can  be  evoked  not  only 
at  the  tendon  above  and  below  the  patella  and  from  the  patella  itself,  but 
also  by  striking  the  periosteum  of  the  tibia  in  a  more  or  less  extended  area. 
In  some  cases  where  the  increased  patellar  reflex  is  evoked,  the  quadriceps 
of  the  other  leg  contracts  also  (crossed  reflex) .  The  highest  degree  of  increase 
is  called  patellar  clonus;  here  the  quadriceps  is  affected  by  more  or  less  rapid 
contractions,  which  last  for  some  seconds,  when,  after  the  muscle  has  been 
relaxed  as  far  as  possible,  one  seizes  the  patella  firmly  with  two  fingers  and 
moves  it  suddenly  towards  the  tibia. 

Foot-clonus  (ankle  clonus)  as  an  expression  of  the  increase  of  the  Achilles 
tendon  reflex  can  be  more  constantly  evoked.  When  the  legs  are  totally 
relaxed  (position  as  above  under  3)  the  examiner  with  his  right  hand  placed 
under  the  sole  of  the  patient's  foot  gives  a  sudden  jerk  upward  towards  the 
sole  of  the  foot,  while  simultaneously  the  left  hand  protects  and  slightly 
fixates  the  leg  proper  at  the  knee  joint.  The  calf  shows  slow  or  more  rapid 
clonic  contractions  accompanied  every  time  by  twitching  movement  of  the 


68  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

foot  downwards.  It  is  important  here  that  the  pressure  of  the  hand  upon  the 
sole  of  the  foot  must  be  relaxed,  as  soon  as  the  blow  at  the  sole  of  the  foot 
has  been  delivered;  the  hand  then  remains  but  lightly  resting  against  it  (but 
may  not  let  the  sole  go) .  As  a  rule,  10  to  20  such  twitchings  appear,  decreasing 
gradually;  in  severe  cases,  however,  the  foot-clonus  may  persist  for  minutes. 
From  this  must  be  differentiated  the  pseudodonus  of  hysterics,  in  which 
either  the  calf  comes  more  nearly  into  a  state  of  tremor  or  is  even  actively 
contracted,  or  some— about  3  to  4— clonic  twitchings  appear  as  expression 
of  a  slight  "reflex  hyperesthesia. "  Hand-clonus,  clonic  flexion  movements 
in  the  flexors  of  the  hand  and  fingers,  which  may  be  evoked  as  the  expression 
of  a  high  degree  of  increase  in  the  reflexes  in  the  upper  extremities  by  a 
sudden  jerk  against  the  relaxed  metacarpus,  from  below  upwards,  with 
fixated  forearm,  is  rarer. 

Increase  of  the  tendon  reflexes  is  found,  as  has  been  stated,  almost  con- 
stantly in  increase  of  the  muscular  tonus  (cf.  this)  therefore  in  all  so-called 
spastic  diseases  (the  highest  degrees  of  it  in  diseases  of  the  pyramidal  tracts), 
besides  this,  also  in  neurasthenia  and  hysteria;  but  here,  as  a  rule,  the 
criterion  of  a  genuine  "clonus"  is  lacking. 

The  decrease  of  the  tendon  reflexes  is  difficult  to  judge.  We  may  say  that 
we  can  speak  certainly  of  a  decrease  in  a  (otherwise  constant)  tendon  reflex 
when  it  is  evoked  but  feebly  even  with  the  assistance  of  Jendrassik's  manoeu- 
vre (reinforcement  method),  or  also,  when  there  is  an  obvious  difference  be- 
tween the  similar  reflexes  of  each  half  of  the  body  and  the  stronger  reflex 
must  be  regarded  as  normal,  not  increased.  By  Jendrassik's  reinforce- 
ment method  we  designate  the  strong  active  tension  on  the  part  of  the  patient 
of  a  muscular  area,  during  the  test  of  the  tendon  reflexes  of  another  area. 
Usually  the  patellar  reflex  is  concerned.  If  presence  of  the  reflex  seems 
questionable  one  has  the  patient  exert  tension  upon  the  musculature  of  his 
arms,  by  pulling  apart  with  all  his  might  his  hands  tightly  interlocked, 
without  letting  them  go  (or  by  having  the  patient  heartily  grip  his  own  hand). 
At  this  moment  the  exciting  blow  upon  the  tendon  is  delivered.  Whether  in 
this  process  it  is  a  matter  of  "paving  the  way"  for  the  reflex,  and  not  of 
merely  distracting  the  attention  of  the  patient  and  thereby  securing  better 
relaxation  of  the  muscle,  is  not  yet  known. 

If  even  with  this  help  the  reflex  can  not  be  evoked,  we  describe  it  as 
absent.  The  absence  of  the  patellar  reflex  (and  almost  as  certainly  that  of 
the  Achilles  tendon  and  triceps  reflex)  are  found  in  diseases  with  lowering  of 
the  muscular  tonus  (tabes),  in  neuritis,  in  narcosis  and  coma,  in  muscular 
atrophies  of  high  degree,  in  rare  cases  also,  apparently  congenital.  Oc- 
casionally there  occurs  " exhaustion "  of  the  tendon  reflexes;  after  long 
testing  they  become  weaker  and  more  difficult  to  evoke. 

Among  the  cutaneous  reflexes  only  three  are  of  great  importance;  the  plan- 
tar, the  cremasteric,  and  the  .abdominal. 


CUTANEOUS  REFLEXES  69 

We  designate  by  plantar  reflex  a  movement  of  the  toes,  which  occasionally 
extends  to  the  metatarsus,  upon  irritation  of  the  skin  of  the  sole  of  the  foot 
(done  best  with  the  handle  of  the  percussion  hammer,  also  with  pieces  of  ice, 
by  pinpricks,  etc.).  The  stimulation  should  be  performed  quickly  and 
energetically;  if  the  patients  are  anxious  it  is  best  to  fixate  the  leg  proper. 
Sometimes  the  reflex  of  certain  parts  of  the  planta  pedis  can  be  evoked  with 
special  ease  (usually  this  is  done  best  from  the  inside  edge). 

The  normal  plantar  reflex  consists  in  an  energetic  plantar  flexion  of  all 
the  toes,  occasionally  with  simultaneous  dorsal  flexion  of  the  metatarsus,  or 
of  the  entire  foot  (flight  reflex) . 

The  reflex  is  not  absolutely  constant  in  the  healthy.  It  is  absent  in 
every  severe  hypsesthesia  of  the  planta,  also  in  severe  cornification,  edema, 
cold;  furthermore  in  sleep  and  in  narcosis — besides  in  some  organic  diseases, 
neuritis,  cerebral  and  spinal  paralyses.  Its  mere  absence — -as  well  as  its 
increase — is  but  rarely  of  great  diagnostic  importance. 

Under  certain  conditions  we  find,  however,  instead  of  the  normal  plantar 
reflex,  an  abnormal  reflex  named  after  Babinski:  the  so-called  Babinski 
toe  reflex.  Here  there  appears  upon  irritation  of  the  skin  of  the  planta  a 
slow  dorsal  flexion  of  the  large  toe,  occasionally,  too,  of  the  other  toes  and 
the  middle  part  of  the  foot  instead  of  the  normal,  rapid  plantar  flexion. 
Here  we  must  emphasize  the  fact,  that  one  uses  at  first  but  weak  stimuli, 
slight  strokes  of  the  percussion  hammer,  etc.,  and  stimulates  various  parts 
of  the  planta,  one  after  the  other.  If  this  is  done  too  energetically,  there 
is  often  a  rapid  withdrawal  of  the  foot  and  an  undecided  wavering  of  the 
toes,  composed  of  extension  and  flexion,  which  can  be  interpreted  only  as 
flight  reflex.  The  Babinski  phenomenon  is  found  normally  only  in  suck- 
lings. In  later  life  it  may  be  regarded  as  a  pathognomonic  sign  of  disease 
of  the  pyramidal  tracts.  As  to  its  origin,  whether  it  is  only  a  modification 
of  the  normal  plantar  reflex  or  is  an  independent  reflex,  opinions  are  still 
divided;  it  is  probably  a  matter  of  a  purely  spinal  reflex,  which  can  appear 
only  after  suppression  of  the  normal  plantar  reflex  which  goes  through  the 
cortex  of  the  brain. 

Oppenheim's  phenomenon  may  be  described  as  to  a  certain  degree 
analogous  to  the  Babinski  phenomenon.  Upon  energetic  stroking  of  the 
skin  of  the  leg  at  the  inner  edge  of  the  tibia,  there  is,  in  the  healthy  person, 
no  reflex  movement  of  plantar  flexion  of  the  toes ;  in  spastic  conditions,  on 
the  other  hand,  there  appears  a  dorsal  flexion  of  the  foot  and  the  toes.  I 
found  the  phenomenon  less  constant  than  the  Babinski,  with  which  it 
generally  appears,  and  its  excitation  frequently  somewhat  irritating  to  the 
patient.  In  the  same  group  belongs  also  Remak's  femoral  reflex:  upon 
stroking  the  inner  side  of  the  skin  of  the  thigh,  there  results  reflex  raising 
of  the  entire  leg  and  dorsal  flexion  of  the  foot — usually  only  in  pyramidal 
tract  lesions  of  the  spinal  cord.     Probably  in  all  these  "pyramidal  tract" 


7o  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

reflexes,  a  synergism  of  certain  groups  of  muscles  (extensors  of  the  foot  and 
toes,  flexors  of  the  leg  proper,  ileopsoas)  appearing  only  in  pathological 
cases,  plays  the  main  role;  it  may  be  tested  in  another  way,  too  {StrumpeW  s 
tibialis  phenomenon) :  the  patient  is  asked  to  draw  his  thigh  energetically 
up  to  his  trunk  while  the  examiner  exerts  strong  resisting  pressure  upon  the 
thigh;  in  certain  diseases  (according  to  Strumpell  especially  in  multiple 
sclerosis)  the  synergism  mentioned  appears  especially  in  an  energetic 
contraction  of  the  m.  tibialis  anticus. 

Furthermore  there  occur  occasionally,  also  in  mere  increase  of  the  usual 
plantar  reflex,  co-movements  in  the  musculature  of  the  thigh  of  the  same, 
sometimes,  too,  of  the  other  extremity  and  even  plantar  reflex  on  the  side 
that  has  not  been  stimulated. 

The  cremasteric  reflex  consists  in  a  prompt  contraction  of  the  cremaster 
muscle,  that  is,  an  elevation  of  the  testicle  on  the  side  in  question,  occasionally 
too  on  the  opposite  side,  upon  stroking  (prickj  cold)  the  inner  aspect  of 
the  thigh  near  the  scrotum.  The  reflex  which  in  the  female  is  replaced  by 
the  analogous  inguinal  reflex  and  is  rather  constant,  is  important  only  when 
there  is  some  difference  between  the  two  sides  and  in  total  absence,  especially 
in  transverse  diseases  of  the  spinal  cord,  in  which  the  knowledge  of  the  loca- 
tion of  a  reflex  arc  is  of  importance  for  exact  local  diagnosis. 

The  same  is  true  of  the  abdominal  reflexes.  We  know  of  three  such  on 
each  side — the  upper  or  epigastric,  the  middle  and  the  lower  (hypogastric) 
reflex.  They  are  evoked  by  transverse  stroking  of  the  upper,  middle,  or 
lower  third  of  the  skin  of  the  abdomen  and  consist  in  a  sudden  contraction 
of  the  obliqui  and  the  transversus  abd.  In  most  cases  they  can  be  evoked 
separately;  but  frequently  they  are  altogether  lacking  when  the  abdominal 
walls  are  very  flaccid  or  very  thick  (fatty,  edematous).  Their  absence, 
moreover,  may  be  considered  an  early  symptom  of  multiple  sclerosis.  The 
absence  of  separate  abdominal  reflexes  may  attain  great  importance  for 
localization  in  myelitic  processes. 

We  must  mention  briefly  the  almost  constant  anal  reflex  (contraction 
of  the  sphincter  ext.  upon  pricking  or  a  similar  irritation  of  the  skin  of 
the  anus)  and  the  scrotal  reflex,  the  peculiar  slow  contraction  of  the  tunica 
dartos  of  the  scrotum,  which  upon  stroking  or  refrigeration  (uncover- 
ing) passes  in  curious  waves  over  the  scrotum.  The  latter  is  remarkable 
because  at  least  one  of  these  reflex  paths  might  pass  through  sympathetic 
fibres.  These  two  reflexes  have  not  yet  attained  any  clinical  importance 
worth  mentioning. 

In  respect  to  the  mucous  membrane  reflexes  we  can  be  very  brief.  Only 
the  corneal  or  eyelid  and  the  vomiting  or  retching  reflex  are  important.  In  the 
eyelid  reflex  there  appears  upon  touching  the  conjunctiva  or  cornea  immediate 
contraction  of  the  orbicularis  (to  be  distinguished  from  the  reflex  closure  of 
,the  orbicularis  upon  touching  the  lashes,  which  is  probably  to  be  considered 


INTERNAL  REFLEXES  71 

a  cutaneous  reflex),  and  the  eye  closes.  The  vomiting  reflex  consists  of  a 
retching  movement  produced  reflexly  by  touching  the  posterior  wall  of  the 
pharynx.  Both  reflexes  are  occasionally  found  to  be  changed  (lacking)  in 
organic  processes  in  the  brain  and  the  medulla  oblongata.  But  besides 
this,  they  are  frequently  lacking  in  psychogenous  affections  (hysteria);  but 
since  they  are  not  constant  even  in  the  normal  individual  and  (especially 
the  vomiting  reflex)  can  be  suppressed  to  a  great  extent  by  will-power,  the 
title  of  hysterical  stigma  has  been  unjustly  assigned  to  them. 

Of  the  internal  reflexes  we  have  already  discussed  one,  the  pupillary. 
But  we  must  mention  here,  furthermore,  the  reflex  processes  subserving  the 
evacuation  of  feces  and  urine  as  well  as  those  of  the  sexual  sphere.  It  is  true 
that  we  have  no  real  test  for  these  reflexes;  but  the  pertinent  statements  of 
the  patients  and  the  clinical  observation  of  the  physiological  course  of  these 
processes  permit  of  a  rather  accurate  representation  and  the  leading 
sphincter  disturbances  ought  not  to  be  neglected  in  any  general  nerve  ex- 
amination. 

Evacuation  of  urine  and  feces  is  indeed  subserved  by  both  active  and 
reflex  processes.  The  strong  desire  to  evacuate,  which  occasionally  even  in 
the  normal  individual  overcomes  the  resistances  of  the  sphincters,  is  certainly 
for  the  most  part  reflex,  caused  by  the  degree  of  fulness  in  the  intestines  or 
the  bladder  or  by  the  manner  of  filling  (irritation  of  the  walls  by  pathologically 
changed  contents).  The  disturbances  of  these  functions  are  called  incon- 
tinentia alvi  (urinae),  that  is,  the  inability  to  hold  back  the  contents,  and 
retentio  urinae  (retentio  alvi),  the  impossibility  of  active  evacuation.  Natu- 
rally these  names  are  also  used  promiscuously  for  the  disturbances  of  the 
active  powers  concerned  for  voluntary  evacuation.  Still  we  may  regard  the 
retention  phenomena,  such  as  are  found  in  cerebral  and  high  lesions  of  the 
spinal  cord,  as  well  as  incontinence  in  destructions  in  the  lower  segments  of 
the  spinal  cord,  with  some  justification,  as  a  disturbance  of  the  reflex  of 
evacuation  of  urine  and  feces. 

The  reflexes  of  the  sexual  sphere  are  only  of  slight  importance  for  the  diag- 
nosis of  organic  diseases,  with  the  exception  possibly  of  the  phenomenon  of 
impotence  (cceundi)  in  tabes  and  possibly  as  a  rare  symptom  of  the  phenom- 
ena of  functional  loss  in  diseases  of  the  lowest  segments  of  the  spinal  cord, 
analogous  with  the  animal  experiments  of  L.  R.  Mutter  (loss  of  ejaculation  in 
disturbances  of  the  sacral  portion  of  the  cord).  All  the  greater  is  the  im- 
portance of  the  numerous  psychogenous  disturbances  which  belong  to  this 
realm,  such  as  psychic  impotence,  priapism,  too  frequent  pollutions,  etc. 
Especially  in  the  various  forms  of  neurasthenia  (cf.  in  the  special  part)  do 
we  meet  those  reflex  disturbances,  to  which  strictly  speaking,  many  dis- 
turbances in  menstruation  also  belong.  It  is  common  to  all  internal  reflexes, 
with  the  exception  of  the  pupillary  alone,  that  the  paths  of  the  sympathetic 
nervous  system  play  an  important  role  in  producing  them. 


72  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

F.  Vasomotor,  Trophic,  and  Secretory  Disturbances 

To  this  chapter  belong  the  least  investigated  disturbances  of  the  entire 
neurological  field.  This  is  true  especially  of  vasomotor  phenomena  of  the 
origin  of  which  we  know  only  that  they  possess  centers  in  the  medulla  oblon- 
gata and  their  paths  for  the  most  part  or  altogether  are  the  sympathetic 
tracts;  disturbances  of  this  kind  are  found  in  neuroses  (partly  independent) 
and  in  diseases  of  the  cervical  sympathetic,  in  the  latter  case,  in  a  definite 
arrangement.     As  concomitant  symptoms  of  organic  diseases  they  are  rarer. 

Abnormal  redness  or  pallor  of  the  skin,  abnormal  warmth  or  cold  are 
naturally  to  be  considered  disturbances  only  when  they  follow  inadequate 
stimuli.  Occasionally  such  a  condition  of  abnormal  redness,  pallor,  coolness, 
persists  for  days  and  weeks,  apparently  caused  by  a  paralysis  or  a  spasm  of 
the  vaso-constrictors.  The  most  typical  form  of  such  local  vascular  dis- 
turbances is  Raynaud's  disease  (symmetrical  gangrene),  the  incipient 
stages  of  which  are  mostly  characterized  by  pallor  and  coldness  of  the  end 
phalanges  which  later  passes  into  gangrene.  Similar  conditions  of  tempo- 
rary nature  play  the  main  part  in  intermittent  claudication  (dysbasia  or 
claudicatio  intermittens  arteriosclerotica),  where  through  vascular  spasm, 
pallor  and  coldness  of  the  skin,  pains  and  disturbances  in  movement  appear. 
From  this  one  must  differentiate  the  akinesia  algera  (Mobius),  general 
motility  disturbance  upon  a  foundation  of  psychogenous  pains.  Also  in 
abnormal  redness  due  to  vascular  paralysis  (or  dilator  spasm)  pains  may 
appear  in  rare  cases  (erythromelalgia).  Occasionally  these  vascular  dis- 
turbances are  unilateral,  for  instance,  in  the  face  in  disease  of  the  cervical 
sympathetic. 

Dermographism  (urticaria  factitia)  is  to  be  understood  as  abnormal 
irritability  of  the  vasomotors,  as  it  is  found  in  some  neuroses  and  especially 
in  meningitis;  upon  rapid  stroking  of  the  skin  (finger  nail,  percussion  ham- 
mer) there  appears  upon  the  spot  stroked  a  temporary  reddening,  then  an 
anaemic  white  swelling  which  persists  for  minutes  and  even  hours,  possibly 
caused  by  exudation  into  the  tissue. 

These  serous  exudations  often  appear  in  the  form  of  local  or  general 
edemas  which  are  distinguished  from  those  in  renal  disease  only  by  the 
normal  findings  in  the  circulatory  apparatus,  as  well  as  by  their  transitory 
nature;  in  older  cases  also  by  their  great  obstinacy.  There  has  been  de- 
scribed as  intermittent  edema  an  independent  "vasomotor"  neurosis  in  which 
a  local  edema  appears  now  here,  now  there,  in  the  skin  or  mucous  mem- 
branes, lasting  some  hours  or  even  days.  The  joints  also  may  be  affected 
by  similar  disturbances  (hydrops  articular,  intermittens).  In  hysterics  we 
distinguish  the  so-called  blue  edema  (with  vascular  congestion)  and  white 
edema  (with  anaemia)  which  mostly  appear  locally,  unilaterally,  and  may  be 
very  obstinate. 


TROPHIC  DISTURBANCES  73 

The  trophic  disturbances  appear  in  more  striking  form  than  the  vasomotor. 
Besides  the  muscular  atrophy  already  discussed,  the  skin  and  skeleton  fre- 
quently exhibit  trophic  disturbances  dependent  upon  nervous  diseases. 
Some  form  a  borderland  between  neurology  and  dermatology.  Among 
these  I  reckon  scleroderma,  in  which  circumscribed  or  diffuse  parts  of  the 
skin  (especially  forehead,  nose,  fingers)  through  loss  of  elasticity  and  shrink- 
ing of  the  skin  become  smooth,  glossy,  usually  brownish  in  color;  the  skin 
can  no  longer  be  raised  from  the  parts  beneath  and  considerable  restrictions 
in  movement  result.  Furthermore,  herpes  zoster,  a  blister-like  eruption 
upon  certain  segments  of  the  skin,  accompanied  with  neuralgiform  pains, 
ichthyosis,  usually  a  congenital  disease  characterized  by  abnormal  dryness 
and  desquamation  of  the  skin,  the  cutaneous  atrophy  known  as  "glossy 
skin,"  in  spinal  cord  and  nerve  trunk  disease,  myxedema,  in  which 
besides  a  diffuse  swelling  and  pallor  of  the  skin  (though  upon  pitting 
the  depressions  do  not  persist),  trophic  disturbances  in  the  nails,  hair,  etc., 
may  also  appear.  Trophic  disturbances  of  the  hair  are  by  no  means  in- 
frequent; loss  of  hair  in  spots  (alopecia)  and  sudden  diffuse  or  circumscribed 
canities  are  the  main  types,  both  of  which  frequently  appear  after  or  because 
of  nervous  diseases. 

The  most  severe  forms  of  atrophic  skin  affections  are  the  ulcers,  among 
which  we  cannot,  of  course,  include  the  artefacts  of  hysterics  who  occasion- 
ally produce  ulceration  themselves.  But  there  does  belong  here  the  mat 
perforant  of  syringomyelia  and  tabes,  the  spontaneous  occurrence  of  deep, 
sharp-edged  ulcers,  especially  on  the  planta  pedis,  and,  furthermore,  the 
already  mentioned  Raynaud's  disease  in  its  last  stages.  In  a  certain  sense 
we  must  include  here  also  the  extensive  decubital  sores  (due  to  pressure  upon 
anaesthetic  places)  of  myelitics  and  the  mutilation  of  the  phalanges  in  the 
so-called  "Morvan's  type"  of  syringomyelia. 

Trophic  disturbances  in  the  bones  and  joints  are  seen  in  the  form  of 
atrophies  as  well  as  of  hypertrophies.  The  former  appear  especially  in 
spinal  diseases  accompanied  by  muscular  atrophy,  as  in  infantile  spinal 
paralysis,  where  usually  the  bone  of  the  extremity  affected  remains  behind 
in  growth  as  the  musculature  disappears;  hypertrophies  form  the  charac- 
teristic of  acromegaly,  where  the  thickening  of  the  bones  (especially  in  the 
forehead,  chin,  fingers  and  toes)  besides  the  thickening  of  the  other  tissues 
is  the  substratum  of  the  disproportionate  condition  of  the  "acra,"  the 
ends  of  the  extremities.  Destruction  of  bones  is  found  in  spontaneous 
fractures  and  especially  in  arthropathies  as  in  severe  spinal  diseases  (tabes, 
syringomyelia).  Here  there  appear  painless  inflammations  of  the  joints 
accompanied  by  hydrops  and  pronounced  thickening,  later  destruction  of 
the  capsule  and  the  articular  ends  of  the  bones,  which  in  their  turn  may 
lead  to  fractures,  luxations  and  subluxations  (Fig.  25). 

The  secretory  disturbances  form  the  smallest  group  of  this  division.     If 


74 


GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 


we  do  not  include  among  them  the  above  described  disturbances  in  evacua- 
tion of  the  feces  and  urine,  only  abnormal  conditions  in  secretion  of  sweat 
and  saliva  are  concerned.  We  observe  occasionally  an  abnormal  dryness 
of  the  mouth  in  tabetics,  abnormal  increase  in  saliva  (salivation,  ptyalism) 
in  epileptics,  affections  of  the  sympathetic,  etc.     The  sweat  secretion  may 


Fig.  25, 


-Arthropathy  and  hypotonia  (genu  recurvatum  in  tabes  dorsalis). 
Krieger,  Klinischer  Atlas  der  Nervenkrankheiten.) 


{After  Schoenborn- 


occasionally  be  present  in  the  form  of  a  defect  {anhidrosis)  in  the  above-men- 
tioned cutaneous  affections  (ichthyosis),  and  may  occur  also  in  some  forms  of 
neuritis  and  in  spinal  diseases.  More  common  is  hyperhidrosis  in  all  possi- 
ble organic  (syringomyelia,  neuritis)  and  especially  in  "functional"  nervous 
diseases  (neurasthenia,  Basedow's  disease);  frequently  it  is  unilateral, 
corresponding  to  the  localization  of  the  process  and  not  rarely  very  easily 
influenced  by  the  psyche. 


EXAMINATION  OF  THE  CEREBROSPINAL  FLUID  75 

Of  all  the  above-mentioned  disturbances  of  vascular  innervation  and 
of  the  trophic  centers  we  can  control  the  occurrence  only  of  a  very  few.  For 
one  single  group  of  these  symptoms  there  are  more  favorable  conditions, 
i.e.,  disease  of  the  cervical  sympathetic.  We  know  clinically,  in  addition  to 
the  experimental  results  of  stimulation  and  total  division  of  the  cervical 
sympathetic,  that  in  paralysis  of  the  cervical  sympathetic,  contraction  of  the 
palpebral  fissure  and  the  pupil  on  the  same  side,  occasionally,  too,  constric- 
tion of  the  cutaneous  vessels  and  anhidrosis  on  the  side  concerned  may 
appear.  Frankly,  the  findings  are  not  altogether  constant,  but  the  opposite 
symptoms  which  would  naturally  be  expected  from  irritation  of  the  cervical 
sympathetic  are  still  less  constant. 

G.  Examination  of  the  Cerebro-spinal  Fluid 

This  examination,  especially  since  the  increase  in  epidemic  meningitis, 
should  be  familiar  to  the  practising  physician  and  to  the  neurologist.  It  is 
connected  with  the  performance  of  lumbar  puncture,  the  inventor  of  which 
was  Quincke. 

For  lumbar  puncture  we  need  as  instruments  a  sharp-pointed  canula, 
10  cm.  in  length,  of  a  diameter  at  most  of  1  mm.  and  a  suction  tube  con- 
nected with  it  by  means  of  a  rubber  attachment.  During  its  performance 
the  patient  lies  on  his  side  or  sits  on  a  chair,  in  each  case  with  spinal  column 
flexed  far  forward.  The  canula  supplied  with  its  mandril  is  inserted,  with 
all  antiseptic  precautions,  between  the  2  and  3,  3  and  4,  or  4  and  5  lumbar 
vertebrae,  about  1/2  cm.  laterally  from  the  median  line  and  in  direction 
inward  and  upward  (in  children  in  the  median  line  sagitally  upward). 
After  piercing  the  muscles,  the  instrument  meets  in  the  ligaments  before 
and  in  the  foramen  intervertebrale  a  slight,  then  in  the  dura,  a  still  slighter 
hindrance.  Now  the  point  of  the  canula  is  in  the  arachnoidal  sac  which  is 
filled  with  liquor;  the  mandril  is  withdrawn  and  the  liquor  drips  or  spurts 
from  the  canula.  Now  the  suction  tube  and  the  rubber  attachment  may  be 
connected  and  the  pressure  of  the  fluid  be  measured  at  once,  the  pres- 
sure normally  amounting  to  40  to  130  mm.  of  water  and  pathologically  may 
rise  to  several  hundred  millimeters.  The  experienced  may  judge  the  pres- 
sure as  normal,  decreased,  or  increased  from  the  manner  in  which  the  liquor 
leaves  the  canula,  whether  it  be  a  rapid  or  slow  dripping,  or  a  sudden  spurt- 
ing. In  this  case  we  take  for  diagnosis  only  4  to  6  c.  cm.,  though  for  thera- 
peutic reasons,  it  is  true,  often  far  larger  quantities  (up  to  40  to  60  c.  cm.  at 
one  sitting,  especially  in  various  forms  of  meningitis).  Then  the  needle  is 
rapidly  withdrawn,  the  small  wound  closed  with  adhesive  plaster.  The 
operation  (unless,  from  the  restlessness  of  the  patient,  the  needle  swerves 
into  the  periosteum)  is  almost  painless.  On  the  other  hand,  there  appear 
frequently,  especially  in  patients  with  normal  fluid,  a  few  hours  after  the 


76  GENERAL  DIAGNOSTICS  OF  NERVOUS  DISEASES 

puncture,  pains  in  the  nuchal  region  and  the  head,  which  may  be  very 
violent  and  last  for  several  days;  they  never  have  serious  consequences  and 
are  easily  overcome  if  the  patient  will  lie  quietly  on  his  back.  Only  in 
tumors  of  the  posterior  cranial  cavity  are  there  occasionally  after  the  punc- 
ture sudden  displacements  within  the  skull  due  to  change  in  the  pressure 
(closure  of  the  aqueduct)  and  now  and  then  such  a  patient  dies;  in  tumors 
of  this  region,  therefore,  puncture  should  not  be  performed. 

The  fluid  in  its  normal  condition  is  as  clear  as  water  and  contains  only 
minimal  traces  of  albumen  and  very  few  cells. 

Pathological  changes  may  affect  the  pressure,  the  chemical  and 
microscopic  condition  of  the  cerebro-spinal  fluid. 

The  pressure  is  rarely  diminished,  on  the  other  hand,  often  increased 
(in  hydrocephalus,  tumors  and  often  in  meningitis)  as  has  been  stated  above. 

Concerning  its  chemical  condition,  the  albuminous  content  is  of  special 
importance.  Increase  in  the  quantity  of  albumen  (apparently,  in  particular 
of  the  serumglobulin)  is  found  in  purulent  or  hemorrhagic  processes  of  the 
meninges  and  of  the  surface  of  the  brain  and  the  spinal  cord,  furthermore 
in  tubercular  meningitis  and  above  all  in  progressive  paralysis  of  the  insane, 
where  it  may  prove  to  be  an  important  differential  diagnostic  factor;  pure 
globulin  increase  seems  to  occur  besides  these  also  in  other  metasyphilitic 
diseases  of  the  cerebro-spinal  system. 

The  microscopy  of  the  fluid  concerns  its  cellular  content  {cytology)  and 
the  micro-organisms  it  contains.  We  speak  of  an  increase  of  contents, 
especially  of  small  uninuclear  cells  ("lymphocytes")  if  the  number  of  these 
formations  in  the  visual  field,  in  an  enlargement  of  400,  amounts  to  more 
than  4  to  5  in  the  average.  This  holds  good  especially  for  all  metasyphilitic 
diseases  (gummatous  processes,  tabes,  progressive  paralysis  of  the  insane) 
and  for  meningitis  of  all  kinds.  Occasionally,  too,  one  finds  large  quantities 
of  polynuclear  cells  (fresh  meningitis  and  progressive  paralysis).  For  the 
purpose  of  this  examination,  a  drop  of  the  strongly  centrifuged  fluid  is  taken 
from  the  bottom  of  the  centrifugation  glass,  carefully  dried  on  the  object 
slide,  eventually  fixed  and  stained  with  methylene  blue  or  May-Griinwald's 
staining  solution,  avoiding  all  rough  measures  (rough  washing).  It  is 
important  not  to  delay  the  examination.  Of  micro-organisms  there  are 
found  in  meningitis  forms  always  corresponding  to  the  aetiology,  most 
frequently  tubercular  bacilli,  then  meningococcus  intracellularis,  pneumo- 
coccus,  influenza  bacilli,  etc.  The  test  (in  the  centrifuged  fluid  or  in  fluid 
which  has  been  set  aside,  same  staining  as  usual)  may  be  very  troublesome. 
The  examination  of  the  fluid  may  be  of  decisive  importance  especially  for 
meningitis  and  for  cases  of  doubtful  postsyphilitic  diseases.  In  all  pure 
psychoneuroses  and  neuroses  (at  least  when  no  syphilis  appears  in  the  history) 
the  fluid  is  normal. 

Cerebral  puncture  described  by  Neisser,  Pollack,  Pfeiffer  and  others  may 


EXAMINATION  WITH  RONTGEN  RAYS  77 

be  spoken  of  as  an  extension  of  lumbar  puncture.  In  this  a  fine  drill  driven 
by  electricity  is  used  to  pierce  the  cranium  and  then  fine  canulas  may  be 
driven  deep  into  the  substance  of  the  brain  through  the  punctured  hole. 

For  the  diagnosis  of  cysts  and  (by  removal  of  bits  of  substance)  of  tumors, 
the  method  is  unusually  valuable,  presenting  no  danger  worth  mentioning 
to  the  patient;  for  the  present,  however,  its  use  should  still  be  restricted  to 
hospitals  and  clinics. 

H.  Examination  with  Rontgen  Rays 

Unfortunately  the  Rontgen  process  that  has  come  to  be  so  invaluable 
for  internal  medicine,  has  as  yet  been  of  little  use  and  help  in  neurology. 
Indirectly,  of  course,  it  may  be  valuable,  if,  for  instance,  it  proves  the  pres- 
ence of  an  aortic  aneurism  as  causation  of  a  recurrens  paralysis — of  a  bone 
fracture  in  a  neuralgia.  But  for  actual  neurological  diagnostics,  one  should 
try  to  learn  clearly  what  the  method  can  be  expected  to  do;  of  course,  it  is 
out  of  question  to  speak  of  proof  of  columnar  diseases  in  the  spinal  cord, 
neuritis,  diffuse  cerebral  diseases  and  all  neuroses.  The  chances  in  trophic 
disturbances  are  somewhat  better.  In  arthropathy,  mal  perforant,  Ray- 
naud's disease,  acromegaly,  we  can  well  establish  the  hypertrophies  and 
destruction  of  the  bones  upon  the  radiograph.  This  may  be  possible  also 
for  muscular  atrophies.  Tumors  of  the  substance  of  the  brain  or  even  of  the 
spinal  cord  are  unfortunately  only  very  rarely  or  not  at  all  to  be  distinguished 
from  parts  covered  with  osseous  tissue,  which  give  quite  diffuse  shadows. 
Only  processes  in  the  bone  itself,  that  is,  tumors  of  the  base  of  the  skull 
or  the  cranial  cap  with  pressure  upon  or  extension  to  the  brain,  as  well  as 
vertebral  affections  (tumors,  caries)  can  easily  be  made  visible — the  latter 
alas — not  nearly  so  frequently  as  is  to  be  desired  (especially  in  incipient 
cases)  and  only  in  favorable  locations  (lumbar  vertebrae,  sacrum,  cervical 
vertebras).  Proof  of  the  presence  of  foreign  bodies  (bullets)  is  always  an 
easy  and  a  well-repaid  task  of  radiography,  whose  results  deserve  attention 
even  with  regard  to  neuro-therapeutics  (Fiirnrohr). 


II 

DISEASES  OF  THE  PERIPHERAL  NERVES 

BY 

H.  STEINERT  (Leipsic) 


INTRODUCTORY  ANATOMICAL  REMARKS  AND 
DEFINITION 

The  peripheral  cerebro-spinal  nerves  consist  principally  of  fibres  of  the 
peripheral  motor  and  sensory  neurons  (teleneurons).  Morphologically 
and  genetically  the  nerves  of  smell  and  sight  occupy  a  special  position.  Of 
the  other  cerebral  and  the  spinal  nerves  it  may  be  said  that  their  motor 
fibres  emerge  from  the  large  motor  cells  located  within  the  central  organs, 
from  the  anterior  columns  of  the  spinal  cord  and  from  the  nuclei  of  the 
brain  stem,  which  are  comparable  to  the  anterior  horns  of  the  spinal  cord. 
The  sensory  fibres,  on  the  other  hand,  emerge  from  the  spinal  ganglion  cells, 
and  in  the  domain  of  the  cerebral  nerves,  from  the  cells  of  the  ganglia  of 
the  head,  which,  likewise  located  outside  the  central  organ,  are  closely 
related  to  the  spinal  ganglia  in  structure  and  development.  These  are 
the  Gasserian  ganglion;  the  spiral  and  vestibular  ganglia  of  the  eighth 
nerve;  the  superior  and  petrous  ganglia  of  the  glossopharyngeal,  and  the 
jugular  and  nodosum  ganglia  of  the  pneumogastric  nerve. 

Each  spinal  nerve  originates  from  the  spinal  cord  by  two  roots,  the  ante- 
rior and  the  posterior.  Through  the  anterior  roots  the  centrifugal,  chiefly 
motor  conduction,  fibres  emerge  from  the  spinal  cord;  through  the  posterior 
roots  the  sensory  fibres  enter  into  the  spinal  cord.  If  the  roots  themselves 
or  the  spinal  nerve  be  injured  immediately  after  the  roots  coalesce,  the 
most  important  clinical  symptoms — those  of  the  loss  of  function — will  be 
almost  identical  with  those  resulting  from  an  affection  of  the  peripheral 
neuron  within  the  spinal  cord  segment  to  which  it  belongs.  These  clinical 
symptoms  are  therefore  treated  in  connection  with  the  diseases  of  the  spinal 
cord,  in  whose  topical  diagnosis  they  play  an  important  part.  They  also 
play  an  important  part  in  dealing  with  the  diseases  of  the  membranes  of 
the  spinal  cord,  in  which  the  roots  of  the  spinal  nerves  are  imbedded,  so  that 
in  the  main  we  look  upon  these  diseases  as  partial  symptoms  of  diseases  of 
the  spinal  cord  membranes. 

Shortly  after  leaving  the  spinal  cord  the  most  important  spinal  nerves 
undergo  complicated  anastomoses  and  interlacings  of  their  fibres.     Those 

.      78 


PERIPHERAL  NERVES  79 

fibres  belonging  to  the  innervation  areas,  to  the  cutaneous  areas  and  to  the 
single  muscles,  no  longer  are  to  be  found  grouped  in  bundles,  in  which  shape 
they  were  related  to  the  central  organ.  Fibres  of  the  same  spinal  nerve 
roots  separate  into  different  branches  of  the  nerve  plexus;  fibres  of  different 
roots  unite.  In  this  way  there  arises  an  anatomical  basis  for  the  appearance 
of  new  and  distinct  clinical  pictures.  The  symptoms  after  lesion  of  certain 
trunks  of  the  plexus  are  grouped  in  a  characteristic  way,  suited  to  the  new 
anatomical  conditions,  and  differing  from  those  of  the  diseases  of  the 
spinal  cord  roots  or  of  those  of  the  primary  trunks  resulting  through  their 
junction. 

From  these  plexuses  spring  longer  and  shorter  branches,  by  which  the 
nerve  fibres,  now  finally  divided,  are  led  to  their  innervation  areas,  without 
undergoing  any  more  anastomoses.  These  branches,  in  the  narrower  sense 
of  the  word,  may  be  called  peripheral  nerves.  The  clinical  disturbances 
which  result  from  their  lesion  are  again  characterized  by  distinct  groupings 
of  their  symptoms  and  by  local  diffusions.  The  diseases  of  these  nerve 
trunks  and  of  those  of  the  first  named  plexus  form  the  principal  object  in 
dealing  with  the  diseases  of  the  peripheral  spinal  nerves. 

In  the  domain  of  the  cerebral  nerves,  anastomotic  formations  do  not 
play  such  an  important  part  as  they  do. with  the  spinal  nerves.  The  trunks 
proceeding  from  the  brain  retain,  for  the  most  part,  their  essential  composi- 
tion until  their  peripheral  distribution.  Nevertheless,  even  some  of  these 
nerves  possess  anastomoses  in  certain  places  in  their  course,  through  which 
functionally  important  species  of  fibres  leave  or  are  joined  to  them.  In  this 
way  particular  varieties  of  clinical  pictures  are  produced,  depending  on 
whether  the  nerve  trunk  be  injured  in  one  place  or  another,  above  or  below 
an  anastomosis.  These  symptom  complexes  afford  us  important  aid  for 
the  finer  diagnosis  of  the  seat  of  the  lesion. 

A  description  of  the  more  intimate  structure  of  the  peripheral  nerves  is 
unnecessary.  It  is  assumed  that  the  histology  of  the  nervous  fibres,  their 
construction  from  axons,  medullary  sheath  and  neurilemma,  is  known.  All 
fibres  of  peripheral  nerves  are  constructed  after  one  type.  Only  the  olfactory 
nerve  consists  of  non-medullated  fibres.  It  is  further  assumed  as  known, 
that  the  nerve  fibres  are  everywhere  bound  together  to  the  nerve  trunk  or 
branch  by  connective  tissue,  which  contains  also  the  nutritive  blood-vessels. 
According  to  experimental  and  pathological  observations,  the  lymph  spaces 
of  the  nerve  trunk  communicate  with  those  of  the  central  organ.  It  is  a 
widely  accepted  belief  that  the  connective-tissue  capsule  possesses  its  own 
sensory  nerves,  the  so-called  nervi  nervorum. 

Lately  the  question  has  been  discussed,  how  the  functionally  different 
species  of  fibres  are  distributed  in  the  peripheral  nerves,  whether  they  are 
all  mingled  together,  or  whether  certain  fibres  subserving  special  functions, 
occupy  special  places  in  the  nerve  trunk.     The  adherents  of  this  latter  theory 


8o  DISEASES  OF  THE  PERIPHERAL  NERVES 

have  spoken  of  a  sy sterna tization  of  the  peripheral  nerves.  No  conclusive 
results  have  been  obtained  so  far  and  our  answers  to  clinical  pathological 
questions  have  not  been  enriched  from  this  source. 

What  has  preceded  makes  it  clear  that  the  diseases  of  the  peripheral 
nerves  have  been  defined  as  partial  diseases  of  both  peripheral  neurons. 
From  what  follows,  it  will  be  seen  that  a  rather  different  definition  is  somewhat 
more  fitting,  especially  since  the  histological  examination  of  some  cases 
shows,  that,  indeed,  not  only  certain  parts  of  the  neurons  are  affected,  but 
that  these  in  their  total  outspreading  are  anatomically  changed.  We  there- 
fore define  the  diseases  of  the  peripheral  nerves,  as  such  diseases  of  the  per- 
ipheral neurons  as  definitely  start  from  certain  primary  points  within  the 
domain  of  the  peripheral  nerves. 

Classification  of  Peripheral  Nerve  Diseases 

We  divide  these  diseases  into  two  large  groups:  the  destructive  and  the 
neuralgic  diseases.  The  first  group  is  marked  by  a  decided  underlying 
anatomical  condition  consisting  of  serious  disturbances  in  the  structure  of 
the  tissues  and  clinically  through  the  preponderance  of  symptoms  of  lost 
function  (Ausfallerschemungen) .  In  the  neuralgic,  on  the  other  hand,  we 
find  no  regular  underlying  anatomical  condition,  while  peculiar  pains  char- 
acterize the  clinical  picture. 

(I)  THE  DESTRUCTIVE  DISEASES  OF  THE  PERIPHERAL  NERVES 

A.  General  Part 

(i)  General  Morbid  Anatomy 

The  histological  changes  in  destructive  diseases  of  the  peripheral  nerve 
concern  the  nerve  fibres  and  the  nerve  connective  tissue.  The  change 
of  the  nerve  fibres  may  be  divided  according  to  two  primary  types.  One 
form  may  be  designated  as  Wallerian  degeneration.  Since  the  investigations 
of  Waller  (1856)  we  know  that  after  complete  division  of  a  nerve  fibre,  its 
peripheral  part  rapidly — in  fact,  within  a  very  few  days — undergoes  a 
peculiar  degenerative  process.  After  some  finer  changes  in  the  structure 
of  the  axon  have  taken  place,  the  nerve  fibre  degenerates  by  a  kind  of  seg- 
mentation into  a  number  of  fragments,  which,  at  first  larger,  gradually  dimin- 
ish in  size.  An  increase  in  the  mass  of  their  protoplasm  and  the  number 
of  their  nuclei  is  produced  in  the  cells  of  the  myelin  sheath.  The  fragments 
of  the  ruined  fibres  are  gradually  absorbed;  nevertheless  medullary  detritus 
is  found,  even  after  the  expiration  of  some  months,  in  the  myelin  sheath 
(Fig.  26). 

Investigation  of  the  other  important  form  of  fibre  disease  begins  with 


PERIPHERAL  NERVES 


81 


1-0:1 


*       0 


■'-J 


Fig.  26. — Wallerian  degeneration.  (After 
Lugaro.)  Bundle  of  nerve  fibres  from  the  ischi- 
adicus  of  a  rabbit  which  was  constricted  four 
days.  Osmium  preparation.  A ,  place  of  con- 
striction; B,  central  section;  C,  peripheral 
section. 

Fig.  27. — Degeneration  of  fibres  of  the  type 
of  periaxial  medullary  disintegration.  (After 
Stransky.)  Experimental  neuritis  through  lead- 
poisoning.  The  disintegrated  parts  of  the  med- 
ullary sheath  blackened  by  osmium. 


bra 


4 


Fig.  27. 


82  DISEASES  OF  THE  PERIPHERAL  NERVES 

Gombault's  work  in  1880.  We  designate  this  form  with  reference  to  its 
most  pronounced  trait  as  the  type  of  periaxial  medullary  disintegration.  The 
normal  medullary  sheath  of  the  axon  shows,  at  regular  short  intervals,  inter- 
ruptions in  its  course.  At  these  points  the  nerve  fibres  have  waist-like  con- 
strictions, known  as  the  nodes  of  Ranvier.  Here  the  periaxial  medullary 
disintegration  frequently  originates,  first  in  the  immediately  adjacent 
sections ;  the  outer  layer  of  the  neurilemma  is  disintegrated  into  more  or  less 
fine  grains.  This  fine-grained  disintegration  then  seizes  upon  larger  sections 
of  the  fibres  and  pushes  through  the  entire  thickness  of  the  medullary 
sheath.  Here,  too,  by  a  process  of  proliferation,  the  cells  of  the  neurilemma 
participate  in  the  morbid  process.  Histologically,  the  axon  undergoes 
changes  only  later.  Whether  the  total  destruction  of  the  axon,  and  through 
this  the  local  degeneration  of  the  peripheral  part  and  secondary  Wallerian 
degeneration,  is  possible,  is  still  under  debate,  but  there  is  much  in  favor  of 
this  assumption.  Repeatedly  normal  fibre  sections  between  diseased  sec- 
tions have  been  observed  in  this  form  of  diseased  nerves  (segmentary 
neuritis)  (Fig.  27). 

While  Wallerian  degeneration  dooms  the  fibre  to  destruction,  the  changes 
in  periaxial  disintegration  permit  of  a  regeneration.  In  contradistinction 
to  these  two  most  important  forms  of  fibre  disease,  the  simple  atrophying 
processes  play  only  a  subordinate  part  in  the  morbid  anatomy  of  the  clinical 
types  of  these  diseases. 

The  alterations  of  the  nerve  connective  tissues  are  either  secondary  or 
independent.  In  the  first  case,  we  are  concerned  with  a  mere  increase,  a 
sclerosis,  such  as  always  accompanies  the  degenerative  diseases  of  the  paren- 
chymatous tissues.  Less  often  we  find  primary  interstitial  disease,  which 
usually  affect  the  nervous  elements  also.  Especially  those  pure  inflamma- 
tory processes  have  to  be  considered  here,  which  may  arise  in  cases  of 
hyperemia,  exudation,  cellular  infiltration  and  even  with  hemorrhagic  and 
purulent  changes. 

In  the  most  diverse  diseases  of  the  peripheral  nerves  we  frequently  find, 
moreover,  peculiar  changes  in  the  areas  of  the  central  sections  of  the  affected 
neurons.  In  the  first  place,  in  those  diseases  of  the  peripheral  nerves  which 
arise  from  toxic  substances  in  the  blood,  pathological  processes  occur  in  the 
central  organs,  especially  in  the  spinal  cord,  which,  according  to  their  kind 
and  extent,  must  be  regarded  as  independent  complications.  As  a  rule,  no 
clinical  interest  attaches  to  them.  Secondly,  peculiar  changes,  which  are 
evidently  restricted  to  the  central  parts  of  the  peripherally  diseased  neuron, 
may  be  observed  in  many  cases.  Above  all,  the  root  cells  of  the  anterior 
horn  and  the  spinal  ganglia,  are  implicated  in  the  disease.  It  has  been 
ascertained  through  experimental  investigations,  that  usually  a  histological 
change  sets  in  after  complete  division  of  a  nerve  fibre  in  the  appertaining 
ganglia  cell,  which  consists  chiefly  in  the  destruction  of  the  central  Nissl 


DEGENERATION  OF  PERIPHERAL  NERVES  83 

bodies,  situated  in  the  vicinity  of  the  nucleus,  and  in  an  outspoken  eccentric 
displacement  of  the  nucleus.  Analogous  changes  may  frequently  be  noticed 
in  clinical  diseases  of  the  peripheral  nerves.  While  in  most  cases  these 
adjust  themselves  again,  very  different,  undoubtedly  degenerative  changes, 
and  even  total  destruction  of  the  cell  may  take  place,  if  the  nerve  injury  is 
very  serious  and  permanent.  Very  instructive  observations  have  been  made 
on  the  spinal  cords  of  persons  who  have  suffered  amputations.  The  cells 
of  the  anterior  horn,  corresponding  to  the  amputated  extremity,  may 
atrophy  or  may  remain  intact  for  years.  It  is  impossible  to  state  with  any 
degree  of  certainty,  whether  the  one  or  the  other  will  occur. 

The  proximal  sections  of  the  neurons,  as  well  as  the  cells,  may  suffer 
trivial,  or  in  case  of  the  destruction  of  the  cells,  even  considerable  degenera- 
tive changes.  Thus,  for  instance,  dystrophic  processes  are  observed  in 
precisely  those  areas  of  the  posterior  tract  of  the  spinal  cord  which  are 
occupied  by  the  central  continuations  of  the  peripherally  diseased  sensory 
neurons.  Consequently  the  anatomic  process  in  many  cases  presents  itself 
as  a  general  disease  of  the  peripheral  neurons.  Merely  histologically,  it  is 
then  not  always  possible  to  recognize  with  certainty  the  primary  attacking 
point  of  the  injury.  To  decide  in  such  cases  whether  a  primary  peripheral 
disease  exists,  it  is  necessary  to  refer  to  the  clinical  picture. 

Even  if  the  nerve  fibres  are  completely  destroyed,  an  anatomical  and 
functional  regeneration  is  possible,  so  long  as  the  cellular  centers  of  the 
affected  neurons  are  preserved  with  the  central  region.  Whether  the  new 
axon  emanates  from  the  proximal  part  and  therefore,  from  the  central  cell, 
or  whether  it  is  autochthonously  regenerated  in  the  periphery,  is  at  present 
the  subject  of  an  animated  controversy.  Perhaps  the  peripheral  rem- 
nant of  the  destroyed  nerve  does  more  than  merely  serve  as  a  guide  for  those 
fibres  which  newly  originate  from  the  center.  At  any  rate  the  doctrine  of 
the  neurons,  the  theory  of  the  unity  of  the  ganglia  cells  and  their  continua- 
tions, can  not  be  regarded  as  refuted  in  the  chief  points  of  its  embryological 
and  pathological  foundation,  and  this  theory  alone  is  able,  at  present,  to 
explain  satisfactorily  a  great  many  incidents  in  the  pathology  of  the  diseases 
of  the  peripheral  nerve. 

(2)  General  Symptomatology  and  Pathological  Physiology 

The  disturbances  which  are  common  to  the  diseases  of  the  peripheral 
nerves,  are  determined  by  the  nature  of  the  fibres  composing  the  nerve. 
Definitely  known  are  motor,  sensible  and  sensory,  vasomotor  and  secretory 
fibres. 

The  most  important  symptom  of  a  lesion  of  motor  fibres,  is  the  loss  of 
voluntary  motion,  which  may  range  through  all  stages  from  slight  paresis 
to  the  total  loss  of  motion,  paralysis.     Motor  irritation  phenomena  are  com- 


84  DISEASES  OF  THE  PERIPHERAL  NERVES 

paratively  rare  and  have  no  special  practical  interest.  Besides,  it  is  exceed- 
ingly difficult  to  explain  or  to  define  them.  A  disease  of  the  motor  fibres 
should  be  assumed  only  if  clinical  symptoms  of  loss  of  function  are  present. 
In  a  few  rare  cases,  even  if  the  course  of  a  single  motor  nerve,  especially 
the  ulnaris,  is  interrupted  entirely,  there  need  not  be  any  paralysis  in  its 
innervation  area,  although  as  yet  no  experimental  interpretation  or  satis- 
factory explanations  have  been  offered  for  this  occurrence. 

Passive  muscular  contractures  are  observed  to  result  from  peripheral 
paralyses.  In  time  the  antagonists  of  the  paralyzed  muscles  fall  into  a  more 
or  less  extensive  and  permanent  process  of  shortening  and  shrinking,  which 
impedes  also,  to  a  corresponding  degree,  passive  motion  of  the  paralyzed 
group,  and  which  may  lead  to  considerable  deformity.  Another  form  of 
contracture,  which  affects  the  paralyzed  muscles  themselves,  will  be  con- 
sidered when  discussing  facial  paralysis. 

A  detailed  description  of  the  disturbances  of  electric  irritability,  appearing 
in  peripheral  paralysis,  can  be  found  in  this  work  under  the  heading  "Gen- 
eral Diagnostics."  Only  a  few  points,  essential  to  an  understanding  of 
peripheral  nerve  diseases,  are  emphasized  below. 

In  the  lightest  cases  all  disturbances  of  electric  irritability  may  be  lack- 
ing. Often  there  is  a  mere  decrease — this  happens  more  frequently  than 
an  increase  for  any  length  of  time — in  the  electric  irritability  of  the  nerve- 
muscle  area.  In  the  majority  of  cases  a  reaction  of  degeneration  (R.  D.), 
in  one  of  its  numerous  forms,  may  be  shown.  The  sluggish,  worm-like  con- 
traction of  the  muscle  irritated  by  a  direct  galvanic  current,  is  common  to 
all  these  forms.  These  contractions,  according  to  a  generally  accepted 
theory,  are  the  deciding  symptoms  of  R.  D.  It  must  be  borne  in  mind, 
however,  that  sluggish  contractions  also  occur  in  supranuclear  paralyses 
(at  least  in  a  certain  stage  of  the  condition)  occasionally  in  some  of  the 
affected  muscles,  and  that  the  myotonic  reaction  may  also  produce  deceptive 
sluggish  contractions,  which  bear  a  striking  resemblance  to  those  of  R.  D. 

About  the  relation  of  R.  D.  to  peripheral  paralysis,  the  following  should 
be  noted. 

In  some  diseases,  as,  for  instance,  in  lead  poisoning,  R.  D.  has  been 
found  even  in  muscles  not  affected  by  paralysis.  This  fact  may  occasionally 
acquire  significance  as  an  objective  symptom  of  organic  disease  of  the  nerve- 
muscle  apparatus  (which  otherwise  could  not  be  proven  definitely)  or, 
perhaps,  as  an  early  symptom  of  such  disease. 

As  a  rule,  if  paralysis  is  occasioned  by  lesion  of  the  nerve,  the  point  of 
lesion  is  rendered  incapable  both  for  the  transmission  of  volitional  impulses 
and  of  the  electric  stimulus.  If  the  nerve  trunk  is  excited  electrically  above 
the  point  of  lesion,  only  those  nerves  can  contract,  whose  motor  fibres  leave 
the  nerve  trunk  between  the  point  of  lesion  and  the  point  of  irritation.  The 
nerve  section  located  below  the  point  of  lesion,  is  at  first  both  conductive  and 


ELECTRICAL  IRRITABILITY  OF  NERVES  85 

irritable.  After  a  short  period  of  increase  (which,  however,  can  not  always 
be  noted),  its  excitability  decreases  rapidly.  In  the  case  of  a  complete 
division  of  the  nerve  it  disappears  entirely,  approximately,  on  the  twelfth 
day.  The  direct  faradic  irritability  of  the  muscle  is  wont  to  decrease  just 
as  rapidly.  It,  too,  disappears  entirely  in  severe  cases.  The  galvanic 
muscle  irritability  exhibits,  in  the  majority  of  cases,  an  increase  at  the  end 
of  the  second,  or  during  the  third,  week,  and  this  increase  continues  from 
three  to  nine  weeks,  after  which  it  gives  place  to  a  decrease.  The  sluggish 
contractions  occur  during  the  period  of  increase  as  well  as  the  period  of 
decrease. 

The  conditions  of  the  regenerative  period  are  particularly  interesting  in 
those  cases  in  which  the  irritability  of  the  diseased  nerve  branch  is  entirely 
lost.  Voluntary  motion  returns  to  the  paralyzed  area  before  its  excitability 
to  an  electric  current.  Evidently  the  nerve  becomes  able  to  conduct  voli- 
tional impulses  before  it  is  able  to  respond  to  an  electric  stimulus.  In  proper 
cases,  observations  may  be  made  which  show  that  conductivity  for  an  electric 
stimulus  returns  before  electric  irritability.  It  is  found,  for  instance,  that 
when  a  nerve  has  been  completely  divided,  an  electric  stimulus  applied  at 
the  time  of  the  return  of  active  motility  above  the  point  of  lesion,  causes 
the  muscles  to  contract,  while  a  stimulation  below  that  point  is  without 
effect.     The  R.  D.  outlasts  the  paralysis  for  a  considerable  time. 

If  it  were  possible  to  place  the  various  symptoms  of  the  disturbances  of 
electric  irritability,  especially  of  R.  D.,  in  relation  to  certain  physical  or 
chemical  changes  in  the  nerves  and  muscles,  it  would  mean  a  great  step 
forward  towards  the  establishment  of  a  satisfactory  theory  for  these  symp- 
toms. Of  course,  such  knowledge  would  be  of  inestimable  benefit  for  estab- 
lishing a  diagnosis,  and,  above  all,  a  prognosis.  Concerning  the  changes  which 
affect  the  nerve,  it  may  be  said  with  a  tolerable  degree  of  certainty,  that  the 
irritability  depends  on  the  integrity  of  the  medullary  sheath,  while  the  conduc- 
tivity is  dependent  only  upon  the  axon.  The  facts  adduced  above  are  in 
complete  accordance  with  this  view.  The  conditions  during  regeneration 
especially  are  thereby  made  comprehensible. 

If  a  nerve  trunk  is  completely  divided,  the  axon  is  the  first  part  of  the 
destroyed  section  to  regenerate,  by,  it  may  be  assumed,  an  outgrowth  from 
the  central  stump.  The  new  growth  of  the  axon  is  accompanied  by  the 
return  of  conductivity  for  volitional  impulses,  which  was  discussed  above, 
and  of  electric  irritability.  It  is  only  after  a  long  time,  however,  that  the 
newly  formed  nerve  is  clothed  with  a  sufficient  medullary  sheath,  and  it  is 
in  accordance  with  this  that  the  response  of  the  regenerated  section  to  electric 
irritability,  its  electric  stimulation,  returns  much  later  than  its  conductivity. 

It  must  be  assumed,  that  the  altered  irritability  of  the  muscular  substance 
also  depends  on  variations  in  its  more  intimate  structure,  although  no  gen- 
erally recognized  opinions  concerning  the  nature  of  these  variations  exist. 


86  DISEASES  OF  THE  PERIPHERAL  NERVES 

It  is  certain,  however,  that  the  older  theory,  according  to  which  the  coarse 
degenerative  changes  correspond  to  R.  D.,  is  untenable. 

The  muscle  that  responds  with  R.  D.  to  an  electric  stimulus,  responds 
frequently  to  a  mechanical  stimulus — such  as  a  slight  tap  with  a  percussion 
hammer — with  a  sluggish  contraction,  the  so-called  mechanical  R.  D.  In 
this  case,  too,  quantitative  changes  of  irritability  are  found. 

In  a  few  rare  cases,  it  has  been  noted  that  the  paralysis  continues,  not- 
withstanding the  return  of  electric  irritability.  In  many  such  cases  it  must 
be  assumed  that  the  cause  for  the  continuing  deficiency  in  voluntary  motion 
is  not  to  be  sought  in  the  area  of  the  peripheral  neuron.  If,  despite  the  fact 
that  the  neuro-muscular  apparatus  is  evidently  restored,  the  patient  does 
not  regain  control  over  the  muscles  concerned,  it  is  possible  that  the  cause 
may  be  found  in  the  domain  of  psychology.  Oppenheim  has  spoken  of  a 
habit  paralysis.  In  other  cases  this  symptom  is  to  be  explained  differently. 
(See  section  dealing  with  the  course  of  facial  paralysis.) 

The  symptoms  of  loss  of  function  in  the  sensory  area  are  decidedly  less 
important  than  those  in  the  motor  area.  When  a  mixed  nerve  becomes 
diseased,  the  motor  manifestations  of  lost  function  appear  in  the  foreground, 
while  the  sensory  occur  later  and  are  less  pronounced.  They  are  restricted 
to  parts  of  the  innervation  area,  much  more  frequently  than  the  disturbances 
of  motility  (these  partial  disturbances  of  motility  will  be  discussed  later  in 
the  chapter  on  diagnosis).  Occasionally  they  are  missing  entirely,  but  this 
is  very  rare;  especially  rare  during  an  entire  course  of  a  serious  disease. 
Sensory  function  frequently  returns  long  before  the  motor. 

Numerous  attempts  have  been  made  to  explain  these  peculiar  facts, 
but  none  of  them  is  quite  satisfactory.  The  theory  of  the  less  liability  to 
lesion  of  the  sensory  fibres  is  really  no  more  than  begging  the  question.  It 
may  be  taken  for  granted  that  the  double  support  rendered  the  same  sensory 
area  by  different  nerves,  and  anastomotic  formations  between  branches  of 
different  nerves,  play  a  very  important  part,  varying  in  different  individuals. 

The  different  cutaneous  sensation  qualities,  such  as  the  sensory  percep- 
tions of  touch,  pain  and  temperature,  are,  in  most  cases,  equally  impaired; 
but  exceptions  to  this  rule  may  occur,  in  which  cases  the  perceptions  of  pain 
or  temperature  may  suffer  chiefly  or  exclusively. 

Slight  as  is  today  the  basis  on  which  to  found  an  explanation  of  these 
occurrences,  it  is  nevertheless  safe  to  say,  that  they  appear  not  alone  in  dis- 
eases of  the  spinal  cord,  but  also,  though  much  more  rarely,  in  peripheral 
affections. 

Comparatively  frequently,  besides  a  considerable  lowering  or  disap- 
pearance of  tactile  and  thermic  excitability,  hyperalgesia  is  found.  A  slight 
pricking  with  a  needle,  light  pressing  or  stroking  of  the  skin,  or  stimulation 
with  feeble  electric  currents,  even  the  pressure  of  the  clothes,  gives  rise  to 
violent  pains,  while  mere  contacts  are  not  perceived.     Spontaneous  pains 


SENSORY  DISTURBANCES  IN  NERVES  87 

may  also  occur  in  these  cases.  This  condition  has  been  designated  as 
anaesthesia  dolorosa.  It  is  found  often  in  diseases  resulting  from  alcoholic 
or  arsenical  intoxication  and  in  herpes  zoster,  more  rarely  after  injuries  of  a 
nerve. 

Retardation  in  perception  of  pain,  which  is  well  known  in  tabes,  also  occurs 
in  peripheral  diseases.  A  definite  explanation  of  this  phenomenon  is  want- 
ing in  both  cases. 

We  have  just  touched  upon  the  irritation  symptoms.  They  play  a  role 
of  far  greater  importance  in  the  sensory  than  in  the  motor  areas.  An 
actual  hyperesthesia,  it  is  true,  has  not  been  noted  with  certainty  aside 
from  the  previously  mentioned  hyperalgesia.  On  the  other  hand,  par- 
esthesia and  pains  play  a  rather  important  part.  The  first  manifests  itself 
as  formication,  pricklings,  itchings,  chilliness,  and  other  similar  unpleasant 
sensations.  The  pains  are  felt  partly  in  the  course  of  the  nerve,  partly  in 
the  area  it  supplies.  Besides  spontaneous  pains,  tenderness  to  pressure  in 
the  nerve  trunks  plays  an  important,  though  formerly  greatly  overestimated, 
part.  The  pains,  which  during  disease  of  the  nerve  trunk  are  projected 
into  the  area  of  peripheral  distribution,  may  be  traced  to  irritation  of  the 
long  sensory  fibres  which  course  in  the  nerve.  The  nervi  nervorum  are 
probably  largely  responsible  for  the  production  of  the  other  symptoms 
spoken  of.  We  know  scarcely  anything  concerning  the  disturbances  in  sen- 
sation of  the  deeper  parts  (ability  to  distinguish  differences,  of  pressure; 
susceptibility  to  passive  joint-movement)  in  peripheral  diseases.  Especially 
little  is  known  about  the  deeper  innervation  areas  of  the  single  nerves. 

There  occur  cases  of  disease  of  mixed  nerves,  in  which,  contrary  to  the 
above  stated  rule,  the  sensory  loss  of  function  is  greater  than  the  motor  loss. 
Nothing  definite  is  known  about  the  conditions  under  which  this  occurs. 
Sometimes  the  disturbances  in  motility  show  the  character  of  those  present 
in  locomotor  ataxia,  which  according  to  the  theory  established  by  Leyden  is 
caused  by  the  loss  of  centripetal  impulses  (neurotabes  peripherica). 

Since  in  disease  of  the  peripheral  nerves  the  motor  as  well  as  the  sensory 
limb  of  the  reflex  arc  of  the  tendinous,  periosteal,  and  cutaneous  reflexes  are 
affected,  it  is  self-evident  that  disorders  of  the  reflexes  (decrease  and  loss)  will 
occur.  In  general,  one  may  say  that  the  reflexes  cease  very  early.  The 
loss  of  reflexes  may  often  be  the  first  objective  indication  of  a  destructive 
nerve  disease,  just  as  it  commonly  outlasts  all  the  other  disturbances,  and 
may  remain  as  a  last  and  frequently  permanent  residuum.  Cases  occur,  on 
the  other  hand,  in  which  the  reflex,  in  spite  of  an  indubitable  disease  of  the 
area  of  its  reflex  arc,  persists  remarkably  long.  Aside  from  its  occurrence 
as  a  transient  initial  symptom  in  some  cases,  a  reflex  increase  is  an  exceed- 
ingly rare  occurrence,  and  is  always  a  strong  argument  against  a  peripheral 
nervous  disease.  Of  course  only  those  areas  are  meant  which  are  actually 
affected  by  the  disease.     An  increase,  for  example,  of  the  Achilles  tendon  or 


88  DISEASES  OF  THE  PERIPHERAL  NERVES 

patellar  tendon  reflex  in  a  pure  peroneal  paralysis  is  a  common  occurrence, 
as  the  peroneus  nerve  is  not  at  all  essential  for  the  formation  of  these 
reflexes. 

In  a  number  of  infectious  fevers  it  frequently  happens  that  during  the 
febrile  stage,  the  tendon  reflexes  of  the  limbs  disappear,  at  least  temporarily. 
This  can  be  observed  in  lobar  pneumonia,  in  typhoid,  in  diphtheria,  and 
even  in  ephemeral  fever.  The  same  thing  occurs  also  in  a  number  of  toxic 
conditions  (alcoholism,  diabetes  mellitus),  though  in  such  cases  the  loss 
frequently  is  permanent.  It  is  not  yet  known  whether  in  these  cases  the 
anatomic  lesion  is  to  be  sought  in  the  area  of  the  peripheral  nerves. 

We  know  little  definite  about  the  vas"o-motor  disturbances.  It  is 
assumed  that  the  peripheral  nerves  carry  both  vaso-constrictor  and  vaso- 
dilator fibres,  thereby  making  it  possible  to  understand  many  clinical  symp- 
toms which  accompany  peripheral  nervous  diseases.  Here  and  there 
redness  and  heat  are  observed,  in  other  cases  the  diseased  parts  are  cold, 
pale  and  cyanosed,  which  can,  in  part,  be  definitely  ascribed  to  the  disturb- 
ances of  the  innervation  of  nerves.  No  considerable  practical  interest 
attaches  to  these  disturbances. 

Secretory  disturbances  will  be  treated  minutely  in  the  discussion  of 
the  paralysis  of  the  seventh  and  ninth  cerebral  nerves.  In  passing,  only  a 
word  regarding  the  production  of  sweat.  With  the  motor  peripheral 
nerves  course  the  secretory  nerves  for  the  sweat  glands.  Destruction  of 
the  peripheral  nerves  may  lead  to  the  complete  loss  of  sweat  secretion.  In 
the  early  stage  of  peripheral  diseases  and  in  partial  lesions  hyperhidrosis, 
which  may  be  classed  as  an  irritation  symptom,  sometimes  occurs. 

Of  the  trophic  disturbances  those  of  the  musculature  are  by  far  the 
most  important.  Atrophy  of  the  muscle  in  diseases  having  an  acute  onset 
develops  some  time  after  the  paralysis,  while  in  diseases  with  a  more  chronic 
course,  it  may  develop  at  the  same  time  as  the  paralysis.  As  stated  above, 
definite  histological  characteristics  of  atrophy  dependent  upon  disease  of 
the  peripheral  nerves  are  up  to  the  present,  unknown.  The  usual  micro- 
scopic methods  merely  show  the  ordinary  characteristics  of  "simple  atrophy." 
It  may  be  assumed  as  likely,  that  the  trophic  influence  of  the  peripheral 
motor  neurons  coincides  exactly  with  its  functional  significance.  All  irrita- 
tions which  are  transmitted  to  the  muscle  through  a  nerve  tract,  the  psycho- 
motor and  reflex  impulses,  ultimately  take  their  course  over  the  cell  of  the 
anterior  horn  and  the  peripheral  motor  nerves.  If  this  course  is  interrupted 
entirely,  the  muscle  is  cut  off  from  all  nervous  irritation,  while  in  supra 
nuclear  paralysis  or  interruptions  of  the  pyramidal  tract,  irritations  are 
still  transmitted  to  the  peripheral  nerves  through  other  supervening  neurons. 
In  this  way  the  ceteris  paribus,  very  severe,  even  complete,  atrophy  of  the 
muscles  in  many  peripheral  paralyses  is  explained,  in  contradistinction  to 
the  usually  far  less  pronounced  atrophy  in  supra  nuclear  paralysis. 


TROPHIC  DISORDERS  89 

As  to  the  contractures  of  the  musculature  some  preliminary  remarks 
have  already  been  made. 

Of  further  trophic  disturbances,  those  of  the  skin  are  particularly  note- 
worthy. Not  infrequently  the  skin  becomes  peculiarly  glossy,  thin  and 
smooth,  while  the  nails,  on  the  other  hand,  lose  their  lustre,  become  cracked, 
of  irregular  growth  and  sometimes  crooked,  so  that  a  kind  of  onychogry- 
phosis  results.  Occasionally  inclination  to  serious  cutaneous  diseases, 
eruptions,  formation  of  ulcers  and  anomalies  in  hairgrowth  are  observed. 
For  an  explanation  of  edema,  which  is  sometimes  observed,  it  is  necessary 
to  take  into  consideration  the  immobility  of  the  parts  concerned. 

As  a  peculiar  cutaneous  affection  depending  upon  an  affection  of  the 
peripheral  sensory  neurons,  herpes  zoster  is  to  be  mentioned.  It  is  chiefly 
found  accompanied  by  neuralgia  and  will  be  described  briefly  with  it. 

If  the  peripheral  nerves  of  an  entire  extremity  suffer  a  serious  and  ex- 
tended lesion  in  earlier  years,  the  affected  limb  is  retarded  in  growth  in  all  its 
parts.  But  even  in  similar  diseases  of  adults,  acquired  later,  a  slight  degree 
of  bone  atrophy  will  often  be  revealed  by  the  X  rays.  The  joints  suffer 
especially  under  the  influence  of  immobility. 

Besides  the  above  there  are  a  number  of  trophic  disturbances  which 
have  been  designated  as  casuistic  rarities.  They  are  numerous  and  hetero- 
geneous but  no  practical  interest  is  attached  to  them.  In  general,  great 
deviations  from  the  above  types  are  very  uncommon. 

The  essentials  concerning  the  neuro-physiological  origin  of  muscle 
atrophy  have  been  stated  above.  Our  opinion  concerning  the  manner  in 
which  the  other  trophic  disturbances  originate,  is  still  more  pronouncedly 
provisional.  At  all  events,  nothing  forces  us  to  the  conclusion  that  there 
are,  besides  the  other  fibres,  also  trophic  fibres  in  peripheral  nerves  whose 
only  task  would  consist  in  influencing  the  nutritive  condition  of  the  tissues. 
It  is  much  more  likely  that  these  disturbances  are  explained  part  by  the 
injurious  effect  of  the  inactivity  of  the  parts,  more  precisely  by  the  loss  of 
centrifugal  impulses,  partly  by  the  impairment  of  certain  regulatory  an- 
tagonists in  regard  to  external  injuries  on  which  the  integrity  of  centripetal 
tracts  depends.  In  the  first  place  the  cessation  of  sensation  must  be  consid- 
ered. In  fact  many  trophic  disturbances  appear  especially  in  those  diseases 
resulting  in  grave  losses  of  sensory  functions.  True  trophic  changes  in 
joints  occur  only  in  diseases  in  which  there  are  sensor}7  disorders.  Muscle 
atrophy  occurs  also  where  sensibility  is  normal. 

(3)  General  Diagnosis  and  Prognosis 

Destructive  processes  of  the  peripheral  nerves  can,  as  a  rule,  be  diag- 
nosed only  in  those  cases  in  which  clinical  symptoms  of  lost  functions  exist. 
Where  no  paresis  or  paralysis,  no  hypaesthesia  or  anaesthesia  exists,  it  will 


oo  DISEASES  OF  THE  PERIPHERAL  NERVES 

be  very  difficult  to  reach  even  a  partly  certain  diagnosis.  One  should  be 
especially  cautious  in  not  overrating  the  diagnostic  significance  of  pains  and 
tenderness  of  the  nerve  trunks  on  pressure.  The  latter  is  an  equivocal  sign 
and  is  found  under  manifold  circumstances.  In  many  nervous  persons  all 
the  nerve  trunks  react  to  pressure  with  pain  sensations.  In  various  affec- 
tions of  the  joints  the  same  symptom  is  found,  more  or  less  pronounced. 
Very  rarely  pains  appear  in  such  characteristic  ways  and  places  as  to  deserve 
any  diagnestic  consideration.  The  typically  neuralgic  pain,  which  will  be 
considered  in  another  chapter,  does,  indeed,  unquestionably  indicate  a 
disease  of  the  peripheral  nerves,  but  points  to  so-called  neuralgic  changes, 
not  to  destructive  affections.  The  relation  between  neuralgia  and  destruc- 
tive nerve  diseases  will  be  treated  in  the  chapter  on  neuralgia.  In  most 
cases  the  existing  pains  are  not  very  characteristic. 

The  correct  diagnosis  depends  on  the  accurate  interpretation  of  the 
symptoms  of  loss  of  motor  and  sensory  power. 

In  the  first  place,  paralyses  possess  certain  common  traits,  peculiar  to 
the  diseases  of  the  entire  peripheral  neurons,  and  secondly  they  are  charac- 
terized by  certain  extensions  of  the  disturbances  which  correspond  to  the 
innervation  area  of  the  peripheral  nerves.  Taking  these  facts  as  a  criterion 
will  in  many  cases  facilitate  the  diagnosis  considerably. 

The  following  may  be  mentioned  as  general  neuron  symptoms.  The 
peripheral  paralysis  is  in  so  far  absolute  as  the  muscles  not  only  fail  for  cer- 
tain functions,  as  in  many  supra  nuclear  paralyses,  but  are  also  in  the  same 
degree  cut  off  from  all  nervous  stimulating  impulses.  Consequently  the 
so-called  associated  movements,  which  are  frequently  seen  in  central  paraly- 
ses, are  lacking.  A  peripherally  crippled  hand  will  not  reveal  a  contracting 
movement,  even  after  strong  exertion  of  the  healthy  hand. 

In  certain  cases,  a  sort  of  associated  contraction  sets  in  sometimes  in  a 
peripherally  crippled  muscle,  if  intact  nerves  in  its  immediate  vicinity  are 
innervated.  This  constitutes  a  rare,  almost  indefinable  symptom,  which 
has  been  noted  in  the  area  of  the  ophthalmic  muscles.  Another  very 
singular  form  of  associated  contraction  in  paretic  and  paralyzed  muscles 
will  be  spoken  of  in  the  chapter  on  the  course  of  facial  paralysis.  These 
facts,  however,  do  not  destroy  the  value  of  the  above  cited  rule. 

Peripheral  paralysis  is  flaccid.  In  parts  not  wholly  paralyzed,  a  certain 
amount  of  resistance  is  sometimes  noted  in  passive  motions,  if  these  awaken 
pain,  though  a  real  spasm  without  impairment  of  reflexes  is  never  produced. 
The  tendinous,  periosteal  and  cutaneous  reflexes  depending  on  the  diseased 
nerves  are  weakened  or  abolished.  The  genuine  Babinski  reflex  does  not 
occur.  (Concerning  a  pseudo-Babinski  the  reader  is  referred  to  tibial 
paralysis  and  sciatica.)  The  muscles,  at  least  in  the  serious  cases,  become 
very  atrophic.  Electrical  examination  as  a  rule  reveals  R.  D.  But  not  in 
all  cases  will  the  above  mentioned  criteria  permit  of  a  neuron  diagnosis 


DIAGNOSIS  OF  NERVE  TRUNK  DISEASE  91 

with  certainty.  In  the  chapters  of  this  book  dealing  with  the  subject,  it 
will  be  shown  that  central  paralysis  may  also  be  flaccid,  and  be  accom- 
panied by  reflex  losses,  and  that  it,  too,  may  cause  muscle  atrophy  to  a  great 
extent.  On  the  other  hand,  the  muscle  atrophy  in  peripheral  paralysis  may 
be  remarkably  slight,  as  it  is  generally  in  the  lighter  cases.  But  even  in 
severe  peripheral  paralysis,  in  multiple  neuritis  for  instance,  the  muscles  are 
frequently,  from  the  clinical  standpoint,  not  at  all  atrophic,  sometimes  even 
hypertrophic.  In  a  few  cases  it  was  possible  to  trace  the  cause  of  this  con- 
dition anatomically  to  a  lipomatosis  of  the  musculature. 

In  contradistinction  to  these  difficulties,  the  diagnostic  significance  of 
the  extension  of  the  disturbances  to  the  motor  and  sensory  innervation  areas 
of  certain  peripheral  nerves,  in  the  more  restricted  sense,  or  of  certain  branches 
of  the  nerv e  plexus,  by  which  for  one  familiar  with  these  areas  the  diagnosis 
at  once  suggests  itself,  is  all  the  more  important.  More  about  this  may  be 
found  in  the  description  of  the  various  paralyses. 

A  few  special  difficulties  which  may  obstruct  a  proper  diagnosis,  and  are 
particularly  liable  to  mislead  a  novice,  should  be  indicated  here.  We  shall 
not  attempt,  however,  a  discussion  of  the  rather  rare  complication  of  central 
by  peripheral  disturbances. 

If  very  many  peripheral  nerves  are  simultaneously  affected,  the  symptoms 
of  lost  function  may  be  so  extensive  as  to  preclude  the  possibility  of  recogniz- 
ing a  typical  distribution  by  peripheral  areas.  If  entire  extremities,  even  all 
extremities  of  the  body  are  affected,  it  is  yet  possible,  that  by  reason  of  the 
disproportionate  affection  of  the  various  peripheral  nerves  the  peripheral 
distribution  type  may  be  clearly  marked  in  a  few  places,  although  this 
sometimes  is  not  the  case.  Then  the  symptoms  we  have  described  as 
neuron  symptoms,  are  of  predominant  importance  in  determining  the  di- 
agnosis. For  the  diagnosis  of  a  peripheral  disease,  in  contradistinction  to  a 
spinal  affection  of  the  same  neuron,  besides  the  certain  features  of  its  entire 
clinical  picture,  astiological  factors,  condition  of  its  development  and  course, 
which  are  characteristic  of  certain  types  of  diseases,  the  following  is,  above 
all,  important.  Almost  invariably  motor  and  sensory  disturbances  co-exist 
in  peripheral  diseases,  just  as  in  the  most  important  form  of  dissemi- 
nated spinal  affections  of  the  peripheral  motor  neuron,  sensory  disturb- 
ances are,  almost  without  exception,  lacking.  In  other  spinal  diseases, 
besides  symptoms  of  the  peripheral  neuron  disease,  there  appear  typical, 
specifically  central  or  spinal  disturbances,  foreign  to  peripheral  diseases; 
symptoms  of  a  lesion  of  the  pyramidal  tract,  bladder  disturbances,  etc., 
which  unite  with  those  into  characteristic  clinical  pictures  of  the  destruction 
of  certain  spinal  areas.     (See  differential  diagnosis  of  multiple  neuritis.) 

But  even  in  slightly  disseminated  processes  difficulties  may  arise,  since  in 
diseases  of  the  peripheral  nerves  the  symptoms  of  lost  functions  may  be 
confined  to  certain  districts  of  the  innervation  area.    First  the  paralysis  may 


92  DISEASES  OF  THE  PERIPHERAL  NERVES 

exhibit  itself  in  different  branches  of  a  nerve  consecutively,  or  it  may  continue 
to  be  restricted  to  single  branches.  The  preference  which  the  disease 
manifests  for  certain  parts  of  the  nerve  area,  is  in  many  cases  dependent  not 
on  anatomic,  but  on  functional  conditions,  so  that  under  the  influence  of  any 
injuries,  those  parts  will  be  affected  primarily  or  exclusively,  from  which 
especially  strenuous  and  continued  service  is  exacted.  Furthermore,  one 
sometimes  sees  a  predilective  or  exclusive  disturbance  in  the  remotest  part 
of  the  motor  and  sensory  innervation  area  of  a  nerve,  while  the  nearer  parts 
remain  intact.  This  is  said  to  occur  also  in  cases  where  the  lesion  has  doubt- 
less affected  the  main  trunk.  It  has  already  been  noted  that  the  sensory 
disturbances  are  frequently,  almost  regularly,  less  pronounced  than  the  motor. 
It  is  very  seldom  that  sensory  disturbances  are  lacking  completely,  though  it, 
too,  occurs,  and  mostly  in  light  cases.  Many  diseases  of  the  nerve  trunks 
exhibit  only  functional  disturbances  of  certain  fibres  functionally  belonging 
together  and  terminating  in  the  nerve,  whereby  a  "systematizing  tendency" 
is  revealed.  A  classical  example  is  afforded  by  many  cases  of  oculomotor 
trunk  affections,  in  which  only  the  external,  not  the  internal,  eyemuscles  are 
paralyzed.  The  explanation  of  such  occurrences  is  still  a  matter  of  contro- 
versy. After  what  has  been  said,  it  is  obvious  that  it  is  not  always  possible 
to  localize  definitely  intra  vitam  a  disease  of  the  peripheral  nerves.  However, 
those  are  comparatively  rare  exceptions. 

The  prognosis  of  a  peripheral  nerve  disease  must  be  viewed  from 
two  standpoints.  First  with  relation  to  existing  causative  factors.  If  one 
is  certain  that  the  injurious  influences  are  at  an  end,  that  no  new  noxious 
factors  of  any  kind  can  influence  the  diseased  parts,  it  is  possible  to  make  a 
prognosis,  not  conclusively,  it  is  true,  but  with  a  reasonable  amount  of 
certainty,  according  to  the  degree  of  the  existing  disturbances.  A  test  of 
electric  irritability  is  of  great  value  in  such  cases.  Though  one  may  fre- 
quently hit  upon  a  correct  diagnosis  without  such  a  test  yet,  upon  the 
whole,  it  will  be  practically  impossible  to  make  a  fairly  definite  prognosis 
without  careful  electro-diagnosis.  As  stated  above,  an  electric  test  reveals 
the  extent  of  anatomic  destruction.  It  must  be  borne  in  mind,  however, 
that  usually  the  disturbances  of  electric  irritability  are  only  sufficiently 
developed  to  permit  of  definite  judgment,  two,  or  even  three,  weeks  after 
the  injury  has  begun  its  work.  Slight  paralyses  without  any  pronounced 
disturbances  of  the  electric  irritability  may  recover  in  a  few  weeks  or  even 
days.  If  R.  D.  be  present,  however,  it  will  always  require  many  weeks, 
sometimes  even  several  months,  for  complete  restoration.  The  more  the 
electric  irritability  of  the  nerve  trunk  has  decreased,  the  less  favorable  the 
prognosis  becomes.  When  the  nerve  is  mechanically  divided,  the  prospect 
of  reunion  between  the  diseased  and  the  peripheral  part  affects  the  prog- 
nosis considerably.  Concerning  its  importance  more  will  be  said  in  another 
chapter. 


CLASSIFICATION  OF  THE  DESTRUCTIVE  DISEASES  93 

(4)  Classification  of  the  Destructive  Diseases 

The  destructive  diseases  may  be  divided  into  two  large  groups.  One 
group  comprises  those  cases,  in  which  single  peripheral  nerves  are  injured, 
and  those  rarer  cases  of  multiple  disease,  in  which  the  multiplicity  bears 
the  character  of  being  more  of  less  accidental,  so  that  no  definite  system  of 
the  extension  of  the  disturbances  may  be  recognized.  In  a  great  majority 
of  these  cases  a  definite  local  cause  of  the  disease  may  be  proven;  in  the 
remaining  cases,  such  a  local  cause  is  to  be  assumed,  if  the  existing  localiza- 
tion is  taken  into  consideration. 

In  the  second  of  the  main  groups  an  absolutely  certain,  orderly,  more 
or  less  strictly  bilateral,  symmetrical  extension  of  the  disturbances  occurs 
and,  as  a  rule,  the  resulting  typical  course  of  the  disease  is  a  very  pronounced 
feature.  These  clinical  pictures  are  the  result  of  internal  injurious  influ- 
ences of  a  general  character  which  reach  the  nerves  through  the  bodily  fluids. 

It  has  been  repeatedly  attempted  to  make,  besides  this  classification, 
still  another  main  division,  namely,  according  to  pathologic  anatomic  view- 
points, so  as  to  separate  under  the  name  "neuritis"  the  pure  inflammatory 
processes  from  the  rest.  This  attempt  has  proven  a  failure,  and  precisely 
there,  where  we  possess  accurate  pathologic  anatomic  knowledge,  which 
is  not  always  the  case  in  our  field.  We  shall  not  here  point  out  the  diffi- 
culty of  defining  the  "pure  inflammatory,"  but  the  following  must  be 
emphasized. 

In  the  second  main  group  of  our  division,  the  typical  and  regular  altera- 
tion in  the  nerves,  bears,  as  we  now  definitely  know,  a  purely  degenerative, 
but  certainly  not  an  inflammatory,  character.  Yet  we'  find  in  some  cases 
which  neither  clinically  nor  serologically  are  to  be  distinguished  in  any  way 
from  the  others,  besides  the  usual  processes,  purely  inflammatory  foci  in 
several  places.     But  surely  no  subdivision  ought  to  be  based  upon  this. 

From  the  standpoint  of  nomenclature,  therefore,  there  will  scarcely 
be  any  objection  to  the  retention  of  the  term  "polyneuritis"  to  denote  all 
cases  in  this  group,  and  from  the  standpoint  of  language,  too,  the  word 
is  absolutely  correct,  if  one  recalls  the  purely  adjectival  meaning  of  the 
word  neuritis.  As  one  no  longer  thinks  only  of  purely  inflammatory  proc- 
esses when  hearing  the  word  "nephritis,"  so  one  will  also  have  to  deprive 
the  word  "neuritis"  of  the  inflammatory  characteristics,  which  have  fre- 
quently been  assigned  to  it. 

Not  many  attempts  have  been  made  to  subdivide  our  first  group  since 
it  was  realized  that  such  attempts  would  be  impracticable  from  the  view- 
point of  morbid  anatomy  and  would  show  all  the  deficiencies  of  an  artificial 
system,  though  it  has  been  divided  in  such  a  way  as  to  separate  diseases 
resulting  from  acute  mechanical  trauma  as  a  specific  group  from  the  others 
classified  as  "neuritic"  diseases.     But  even  in  this,  some  artificiality  may  be 


94  DISEASES  OF  THE  PERIPHERAL  NERVES 

clearly  detected,  as  will  be  evident  from  the  following  description,  which 
disregards  such  division  entirely. 

In  the  first  place,  therefore,  we  shall  deal  with  the  diseases  affecting 
single  peripheral  nerves,  peripheral  paralysis  in  the  more  restricted  sense, 
mononeuritis  in  the  broadest  sense  of  the  word,  including  also  mono- 
neuritis multiplex,  and  in  the  second  place  we  shall  deal  with  polyneuritis. 

B.  Special  Part 
i.  Diseases  of  Single  Peripheral  Nerves 

General  JEtiology 

Of  greatest  serological  interest  are  the  local  injurious  influences. 
The  relations  to  mechanical  trauma  are  remarkably  diversified.  Mechanical 
injuries  act  directly  or  indirectly  on  the  nerves.  The  direct  traumatic 
effects  are  of  the  most  varied  degrees  from  a  comparatively  light  pressure 
to  complete  division,  tearing  asunder,  cutting  or  crushing  of  the  nerve. 
The  indirect  traumatic  effects  are  produced  by  such  causes,  for  instance, 
as  an  excessive  motion  of  the  body,  producing  an  undue  stretching  of  the 
nerve.  This  will  be  gone  into  more  minutely  when  discussing  paralysis  of 
single  nerves. 

Indirectly,  trauma  may  be  responsible  for  peripheral  paralysis  in  three 
ways.  In  the  first  place,  long  continued  mechanical  irritation,  such  as  the 
pressure  of  crutches,  may  in  time  lead  to  degenerative  processes  and  nerve 
paralysis  (so-called  traumatic  neuritis).  But  even  a  single  mechanical 
insult  seems  to  be  able  to  give  rise  to  similar  diseases.  The  trauma  is  at 
once  followed  by  pain.  Only  after  some  days — or  weeks — the  symptom  of 
lost  function  develops.  Secondly,  conditions  resulting  from  an  originally 
non-nervous  lesion  may  cause  mechanical  nerve  injuries.  After  fracture  of  a 
bone  it  is  sometimes  observed  that  nerve  trunks  not  primarily  affected  may  be 
injured  secondarily  by  bone  fragments.  Scar  tissue  or  the  outgrowth  of 
callus  may  lead  to  direct-pressure  injuries  by  growing  around  and  con- 
stricting a  nerve,  or  nerve  trunks  may  be  forced  into  such  unfavorable  posi- 
tions that  they  are  exposed  to  injuries  whenever  a  member  is  moved.  Nerve 
dislocations,  by  which  the  nerve  is  displaced  from  its  normal  position,  through 
traumatic  influences,  act  in  the  same  way.  Under  such  conditions,  an  old 
trauma  may  lead  to  paralysis  even  after  several  years  (late  traumatic  lesions) . 
In  the  third  place,  finally,  so-called  ascending  neuritis  must  be  considered. 
As  a  result  of  small  infected  wounds,  splinter  injuries,  etc.,  especially  of  the 
hand",  grave  neuritis  of  the  nerve,  in  whose  innervation  area  the  wound  was 
located,  develops  in  rare  cases. 

Other  affections  in  addition  to  trauma  may  cause  mechanical  injuries  to 
nerves.  Tumors,  in  the  broadest  sense  of  the  word,  and  bone  disease,  may 
all  lead  to  paralysis  of  neighboring  nerves  through  pressure. 


CA  USES  OF  LOCAL  NEURITIS  95 

Malignant  tumors  may  also  affect  the  nerve  directly;  inflammation  in  its 
proximity  may  spread  to  the  nerve  itself  and  either  cause  an  inflammation,  or 
its  degeneration  through  toxic  influences.  Ulcers  and  other  cutaneous  dis- 
eases lead  now  and  then  to  degeneration  of  the  sensory  terminal  fibres  in 
their  own  area  and  that  in  their  immediate  vicinity — a  most  interesting  fact 
theoretically,  but  one  which  has  been  but  little  studied. 

In  order  to  explain  a  few. peculiarities  of  the  clinical  picture,  a  separate 
short  discussion  will  be  devoted  to  tumors  arising  from  the  different  an- 
atomical elements  of  the  nerve  trunk  itself. 

A  peripheral  paralysis  occasioned  by  local  chemical  injury  is  sometimes 
seen  after  a  bungling  injection  of  ether  or  camphorated  oil  containing  ether, 
made  in  the  immediate  vicinity  of  a  nerve  trunk.  Some  observations  would 
seem  to  make  it  probable  that  absorption  by  the  skin  of  bisulphide  of  carbon 
— an  injury  to  which  workers  in  rubber  are  often  exposed — may  cause  local 
neuritis. 

Local  thermic  injuries  play  only  a  subordinate  part.  When  discussing 
facial  paralysis  we  shall  meet  with  conditions  which  may  be  regarded  as 
arising  from  local  colds. 

It  is  said  that  arterio-sclerosis  of  the  vasa  nervorum  may  lead  to  degen- 
eration of  the  nerves. 

As  a  final  local  cause  overexertion  of  certain  parts  of  the  body  in 
particular  occupations  must  be  considered.  It  will  be  necessary,  however, 
to  discuss  briefly  the  peculiar  so-called  occupation  paralyses  in  a  separate 
paragraph. 

Besides  the  local  influences,  general  injuries  acquire  some  significance, 
even  in  the  aetiology  of  the  diseases  of  single  peripheral  nerves,  though  it  can 
not  be  said  they  they  play  more  than  a  predisposing  role.  The  same  things 
will  have  to  be  named  here  as  are  enumerated  in  the  aetiology  of  polyneuritis : 
infections  and  toxic  influences,  disorders  of  metabolism,  pregnancy  and  the 
puerperium,  and  debilitated  conditions.  Persons  affected  with  tabes  reveal 
a  peculiar  inclination  to  peripheral  paralysis.  In  all  such  cases  it  is  advis- 
able, however,  to  assume  a  locally  acting  disturbance  besides  the  general  pre- 
disposing causes.  If,  in  a  few  cases,  it  is  not  possible  to  prove  it,  it  should  be 
remembered  that  with  a  strong  predisposition  under  certain  conditions, 
trivial  local  influences  which  may  be  easily  overlooked  suffice  to  excite  dis- 
ease. Sometimes,  at  least,  conjectures  may  be  formed  concerning  their 
nature.  If,  for  example,  an  alcoholic  after  the  passing  of  the  delirium  has 
a  radial  paralysis,  it  can  scarcely  be  amiss  to  assume  a  traumatic  cause  in 
addition  to  the  toxic  predisposition. 

Pathological  Anatomy 

Simple  disintegrative  processes  of  the  nerves  are  much  more  frequent 
than  purely  inflammatory  changes.     The  latter  are  found  especially  in  those 


96  DISEASES  OF  THE  PERIPHERAL  NERVES 

cases  in  which  the  vicinal  inflammation  has  extended  to  the  nerve  (extended 
lymphogenous  neuritis) ,  in  neuritis  emanating  from  infected  wounds  and  in 
local  influences  of  chemical  poisons.  The  nerve  in  such  a  case  is  turgid,  red- 
dish, while  the  microscope  reveals  hyperaemic  and  exudative  changes,  and 
cellular,  even  suppurative  infiltrations.  Soon  also,  naturally,  the  paren- 
chyma participates  in  this  process.  Occasionally  bacteria  may  be  found  in 
the  nerve  trunk.  To  distinguish  between  perineuritic,  interstitial  neuritic, 
or  nodular  forms  according  to  the  degree  to  which  the  perineurium  or  the 
endoneurium  participates,  or  according  to  the  form  of  the  nerve  swelling, 
would  be  useless. 

The  parenchymatous  degeneration  which,  as  was  said,  in  most  cases 
exists  without  actual  inflammatory  complications,  bears  in  most  of  the  lighter 
cases  the  character  of  periaxial  decay.  This  has  been  shown  to  be  true 
especially  of  the  lighter  traumatic  influences.  Though  the  axon  is  surely  not 
anatomically  destroyed,  its  conductivity  for  the  will  impulses,  at  least,  may  be 
affected  temporarily.  In  such  cases  the  mobility  returns  very  soon  after 
the  causative  disturbance  has  been  removed.  Even  if  the  duration  of  the 
injury  be  longer  (as  in  pressure  due  to  callus  and  the  like),  the  disease  may 
remain  in  this  lighter  stage,  and  under  proper  treatment  permit  of  a  very 
favorable  prognosis. 

If  the  local  injury  is  greater,  and  the  axon  is  entirely  destroyed,  the 
peripheral  nerve  section  is  doomed  to  Wallerian  degeneration.  The 
regeneration  will  take  the  longer  the  more  centrally  the  interruption  of  the 
nerve  has  taken  place.  The  reunion  of  the  central  and  peripheral  parts, 
even  if  produced  only  by  interposition  of  granular  tissue  which  paves  the  way 
for  the  newly  formed  fibres  to  the  peripheral  part,  is  an  important  factor 
in  the  process  of  regeneration.  Should  the  reunion  not  take  place,  the  re- 
generative efforts  of  the  central  end  will  lead  to  the  formation  of  what  is 
known  as  amputation  neuromata;  knoblike  swellings  that  form  clinically 
most  irritating  structures,  which  may  cause  violent  pains. 

Those  very  rare  cases  in  which  functional  restoration  has  been  reported 
without  the  reunion  of  the  two  nerve  ends  are  perhaps  best  classed  with 
those  equally  rare  cases,  already  mentioned,  of  the  complete  division  of  large 
nerve  trunks  without  clinical  symptoms  of  lost  function. 

The  clinical  picture  will  be  considered  here  only  very  briefly.  Those 
symptoms  which  occur  in  common  in  destructive  diseases  of  the  peripheral 
nerves  have  already  been  discussed  in  the  general  part  and  the  clinical 
picture  in  diseases  of  different  single  nerves  will  be  shown  in  the  next 
section.  It  may  suffice  to  state  summarily  once  more  here,  that  in  paralyses 
of  mixed  nerves,  the  characteristically  grouped  motor  symptoms  of  lost 
function  together  with  the  accompanying  disturbances  of  the  reflex  function 
and  of  the  electric  irritability,  stand  almost  without  exception  in  the  fore- 
ground.    As  a  rule,  the  sensory  symptoms  of  lost  function,  according  to  the 


DIAGNOSIS  OF  LOCAL  NEURITIS  97 

principle  cited  before,  yield  to  the  motor  ones;  still  they  belong  to  the  cardinal 
symptoms,  even  though  they  must  often  be  sought  for.  More  often 
symptoms  of  sensory  irritation  are  lacking.  No  general  rule  can  be  laid  down 
regarding  their  occurrence  or  non-occurrence.  It  is  to  be  remarked,  however, 
that  their  absence  is  the  rarer,  the  greater  and  more  continuous  the  irrita- 
tion of  the  nerve  trunk  dependent  upon  anatomic  processes  is.  It  would 
be  presumptuous,  nevertheless,  to  draw  from  the  sensory  irritation  symptoms 
definite  conclusions  concerning  the  finer  anatomic  nature  of  the  nerve  disease. 
Least  of  all  is  it  possible  to  establish  a  rule  about  the  pressure  sensitiveness, 
which  sometimes  extends  to  the  musculature  of  the  diseased  region.  In  the 
pure  inflammatory  processes,  cord-like  or  knotty  constrictions  of  the  nerve 
trunk  may  sometimes  be  felt.  Whenever  fever  occurs  it  indicates  that  an 
infectious  process  is  producing  the  paralysis. 

For  a  discussion  of  the  diagnosis  of  the  paralyses  of  single  nerves,  which 
is  usually  very  easy,  the  reader  is  referred  to  the  general  part,  and  for  some 
specific  peculiarities,  to  the  following  section.  Only  a  few  remarks  regarding 
differential  diagnosis  are  made  here.  We  shall  only  mention  here  the  great 
demand  that  complications  blurring  the  clinical  picture  of  the  peripheral 
paralyses,  because  of  central  organic  and  hysteric  disturbances,  make  on 
diagnostic  acumen. 

Real  difficulties  arise  now  and  then,  especially  in  lighter  cases,  when  the 
symptoms  of  the  paralysis  are  not  very  grave,  its  boundary  not  clearly  defined 
and  when  it  affects  only  a  few  of  the  muscles  subserved  by  one  peripheral 
nerve.  Differentiation  of  spinal  affections  of  the  peripheral  motor  neuron 
may  then  be  rather  difficult,  and  all  the  more  so,  because  the  innervation 
areas  of  some  of  the  spinal  segments  are  very  similar  to  those  of  some  of  the 
peripheral  nerves.  Even  when  the  disturbances  of  sensation  do  not  cor- 
respond to  a  whole  peripheral  nerve  area,  but  are  confined,  say,  to  a  distal 
part  of  one,  it  is  not  always  possible  to  base  a  decision  on  this,  as  in  spinal 
diseases  also  the  disturbances  of  sensation  may  be  restricted  to  distal  parts 
of  the  innervation  area.  In  cases  in  which  the  knowledge  of  certain  setiolog- 
ical  factors,  or  the  course  of  the  disease,  or  the  finding  of  decisive  symptoms 
in  other  areas,  does  not  enable  a  sure  diagnosis,  it  will  have  to  remain  in 
abeyance. 

Experience  shows  that  certain  non-neurogenic  contractures,  atrophic 
and  paralytic  conditions,  sometimes  lead  to  an  incorrect  diagnosis,  which, 
however,  might  almost  always  be  avoided  in  these  cases.  Tendon  and 
fascia  contractions,  joint  anomalies  and  cicatricial  processes,  may  produce 
deformations  of  the  limbs,  which  superficially  resemble  the  contracture  pro- 
duced by  peripheral  paralyses.  A  careful  examination  will  enable  one  to 
decide  whether  the  situation  of  the  contracture  is  conditioned  upon  a  loss  of 
function  in  a  definite  nerve  area.  Of  course,  extensive  fixation  of  the  part 
may  make  the  investigation  more  difficult,  especially  in  inveterate  cases,  and 
7 


98  DISEASES  OF  THE  PERIPHERAL  NERVES 

it  will  then  become  necessary,  besides  considering  the  contractibility  of  the 
muscles,  the  reflexes,  the  electric  reaction  and  the  sensibility,  to  consider 
whether  the  existing  deformities  may  at  all  be  traced,  according  to  their  form, 
to  peripheral  paralysis  (for  the  form  of  contractures  in  peripheral  paralysis 
is  usually  very  characteristic) ,  and  whether  other  causes  for  the  deformity  are 
to  be  found. 

It  is  usually  very  easy  to  determine  correctly  arthrogenous  or  arthritic 
atrophy,  because  of  its  characteristic  distribution  in  certain  definite  muscles 
in  the  vicinity  of  a  diseased  joint.  Usually  the  extensors  of  the  joint  are 
affected.  Atrophy  of  the  quadriceps  femoris  in  diseases  of  the  knee  and  the 
predominant  atrophy  of  the  deltoid  and  supra-  and  infraspinatus  in  arthritis 
of  the  shoulder  joint  is  especially  important.  The  functional  impairment 
may  attain  to  actual  paralysis.  The  tendon  and  periosteal  reflexes  are  in 
such  cases  always  exaggerated. 

Ischaemic  paralyses  and  contractures  result  when  the  blood  supply  to  a 
certain  muscle  area  is  seriously  obstructed  by  too  tight  bandages  or,  as  occurs 
more  rarely,  through  lesion  of  blood-vessels.  If  relief  is  not  had  soon,  the 
muscles  fall  into  a  paralytic  and  degenerative  condition  resulting  in  a  rigid 
contracture,  which  is  not  dependent  upon  nervous  lesions.  It  has  been  espe- 
cially noted  in  the  upper  extremities  and  is  minutely  described  in  surgical 
text-books. 

These  and  other  myopathic  paralyses  and  contractures  are  distinguished 
from  similar  peripheral  neuritic  affections,  not  only  by  the  aetiological 
peculiarities,  but  also  by  the  absence  of  pronounced  sensory  symptoms  of 
lost  function,  by  the  regular  absence  of  R.  D.,  and  by  the  peculiar  distribution, 
which  is  not  confined  to  the  innervation  area  of  peripheral  nerves. 

If  careful  consideration  be  given  to  the  nature  of  the  symptoms  of  lost 
function, '  and  if  further,  the  anatomic  extent  of  the  symptoms  which  are 
dependent  upon  the  extent  of  the  innervation  areas  be  as  carefully  determined, 
the  diagnosis  and  differential  diagnosis  will  fail  one  only  in  very  rare  cases. 

Prognosis. — What  was  stated  above  concerning  the  different  aetiological 
factors  will  serve  as  an  ampler  illustration  of  the  fundamental  principle 
already  referred  to  in  the  general  part,  namely,  that  the  prognosis  is  affected 
decisively  by  the  nature  of  the  primary  injuries. 

A  few  viewpoints  not  here  discussed,  will  be  dealt  with  in  the  following 
therapeutic  section. 

General  Therapeutics 

The  prevention  of  peripheral  paralysis  not  infrequently  occupies  the 
attention  of  the  physician.  Both  the  surgeon  and  the  obstetrician  very 
frequently  are  compelled  to  endanger  the  peripheral  nerves  in  various  ways. 
Even  if  it  is  now  and  then,  unavoidable  to  injure  a  nerve  for  the  sake  of 


THERAPEUTICS  OF  NEURITIS 


99 


attaining  more  important  ends,  it  might  more  frequently  be  avoided,  if  only 
the  danger  were  considered.  In  making  hypodermic  injections,  one  ought  not 
to  select  a  place  near  a  large  nerve-trunk.  In  prescribing  medicines,  the 
possibility  of  producing  hazardous  conditions  (as  from  the  too  long  continued 
use  of  arsenic)  must  be  considered.  Of  great  import  is  the  prophylaxis, 
depending  upon  the  hygiene  of  the  trades  or  occupations  in  which  poisons 
are  used.  As  to  predispositions  it  is  necessary  to  remember  the  special 
liability  to  lesion  of  the  nerves  of  these  individuals  with  regard  to  local  in- 
fluences (pressure  paralysis  in  cases  of  tabes  and  in  alcoholics).  These 
suggestions  will  suffice  here.  The  preceding  aetiological  section  furnishes 
some  further  points,  and  in  the  following  chapter  special  cases  will  be 
mentioned,  in  which  the  especial  attention  of  the  physician  is  essential. 

The  actual  treatment  begins  with  an  endeavor  to  remove  the  original 
noxious  factors  as  far  as  possible,  or  to  prevent  their  further  spread.  Further 
procedure  depends  on  the  nature  of  the  injury.  If  a  nerve  is  mechanically 
divided,  surgical  aid,  if  at  all  possible,  should  be  rendered  at  once.  In  other 
cases,  one  should,  in  the  early  stages,  confine  one's  self  to  preserving  in  its 
broadest  meaning  the  diseased  part,  avoiding  all  injurious  influences,  and  to 
symptomatic  alleviation  of  the  most  severe  troubles.  Later,  one's  efforts 
may  be  more  directly  aimed  at  the  preservation  and  restoration  of  function. 
Generally  speaking,  it  may  be  said  that  this  more  active  method  of  proce- 
dure is  ''n  order  only  when  the  regressive  changes  will  positively  not  progress 
any  further.  In  paralyses  caused  by  a  single  acute  insult  this  method  may 
be  started  about  the  third  week. 

General  Measures. — In  the  early  stage  the  diseased  part  should  be 
kept  absolutely  quiet,  be  put  in  slings,  splints,  bandaged  in  cotton,  and  pre- 
served from  shocks  (especially  of  the  nerve  trunks)  by  proper  padding. 
The  warmth  generated  thereby,  is  usually  most  beneficial. 

Anaesthetic  areas  should  be  particularly  guarded.  Mechanical  and 
thermic  injuries,  which  the  patient  does  not  notice  because  of  the  anaesthesia, 
may  lead  to  serious  injuries,  difficult  to  heal  (burns  caused  by  hot-water 
bottles). 

That  the  general  health  must  be  carefully  observed  and  taken  care  of,  is 
not  at  all  merely  a  schematic  phrase,  but  in  our  field,  particularly,  is  of  the 
greatest  importance.  Experience  has  shown  that  the  prospects  for  the 
regeneration  of  destroyed  nerves  depend  considerably  on  the  general  condition 
of  the  patient.  Because  of  this,  general  medical  principles  have  to  be  fol- 
lowed. Anaemia,  disturbances  in  metabolism,  enfeebling  diseases  of  any 
kind,  should  be  treated  carefully ;  the  expenditure  of  strength,  as  far  as  possible, 
reduced,  and  the  diet  regulated. 

Toxic  injuries,  whether  occasioned  by  one's  trade  or  occupation  or  by 
one's  manner  of  living,  should  be  strictly  avoided,  even  if  they  were  not  a 
factor  in  producing  the  disease.     Patients  who  imagine  that  they  may  in- 


Hospital  No.  20, 

ioo  DISEASES  OF  THE  PERIPHERAL  NERVES 

dulge  their  taste  for  alcohol  during  the  treatment,  have  little  prospect  of 
recovery,  and  had  better  be  discharged  from  treatment. 

Medicines  are  to  be  considered  first  for  the  causal  treatment.  It  is 
usual  to  point  out,  that  in  combating  infectious  diseases  and  conditions  due 
to  intoxicative  processes,  it  is  only  necessary  to  proceed  with  suitable  reme- 
dies, such  as  iodides  and  mercury,  for  syphilis;  quinine  for  malaria;  iodide 
of  potassium  for  lead  poisoning.  But  it  is  very  seldom  that  these  factors 
enter  into  the  question  at  all— more  frequently  they  lead,  not  to  paralysis  of 
single  nerves,  but  to  polyneuritis.  Much  more  important  with  us  is  the  use 
of  medicines  with  regard  to  the  individual  symptoms.  To  reduce  the  pains 
we  give  the  numerous  well-known  antipyretic  and  antineuralgic  agents, 
concerning  the  prescription  of  which  the  reader  is  referred  to  the  neuralgias. 
Morphine  should  be  used  very  seldom. 

Besides  various  other  reasons  against  the  use  of  vesicants  and  derivants 
in  painful  conditions,  it  may  be  urged  that  the  injured  skin  may  afterwards 
interfere  with  the  electric  treatment.  If  it  is  desired  to  administer  a  mild 
cutaneous  irritant  beneath  the  bandage,  mistura  oleoso-balsamica  or  a 
10%  menthol  oil  should  be  applied  to  the  aching  parts.  If  an  intense  in- 
flammatory swelling  occurs  near  a  nerve  trunk,  an  attempt  at  relief  may  be 
made  by  applying  a  few  leeches.  After  the  acute  stage  has  ended,  alcoholic 
and  other  embrocations  may  often  be  recommended  in  combating  the  pares- 
thesia.    (Spirit,  camphorat,  5%  menthol  in  alcohol  et  al.) 

Of  those  remedies  to  which  a  tonic  action  upon  the  nervous  system  is 
ascribed  and  from  which  for  that  reason  a  direct  action  upon  the  regenerative 
processes  is  to  be  expected,  preparations  of  strychnine  may  be  tried  first. 
They  are  best  given  in  the  shape  of  so-called  tonic  pills  (Erb). 

T}.    Yen:,  lactic, 

Extr.  chin,  spirit aa  6  .o  (gr.  90) 

Extr.   nucis  vom 0.6  (gr.  9) 

F.  pill.     LX  S.     3  times  daily  a  pill. 

Or  strychnin,  nitrat.  may  be  given  by  hypodermic  injection,  at  first  three 
times  a  week,  after  that  once  daily  i  mg.  The  dose  may  gradually  be  some- 
what increased. 

Hydro-  and  thermotherapy  have  an  extensive  field  of  usefulness.  In 
the  acute  stage  Priessnitz's  compresses,  which  will  usually  have  to  be  applied 
warm,  may  be  tried  alternating  with  dry  dressings.  Other  forms  of  local 
devices  which  retain  the  heat  may  be  tried.  Poultices,  cataplasms,  thermo- 
phores will  very  often  have  a  favorable  action  upon  the  pains.  Cold  appli- 
cations are  not  so  often  beneficial.  Should  this  be  the  case,  water  or  ice  bags, 
ice  cataplasms  and  especially  compress  bandages  may  be  applied,  in  which 
the  limb  is  bandaged  with  a  moist  linen  bandage,  which  is  kept  wet  by  fre- 
quent moistenings.     Protracted  cold  applications  are  in  general  to  be  re- 


THERAPEUTICS  OF  LOCAL  NEURITIS  101 

garded  merely  as  a  transiently  admissible  symptomatic  treatment  in  the 
acute  stage. 

Baths  of  the  entire  body  or  of  the  diseased  part  may  be  used  advan- 
tageously in  any  stage  of  the  disease.  Baths  of  so-called  "indifferent  tem- 
perature" (960  F.,  350  C.)  have  a  quieting  effect  upon  sensory  irritative 
conditions,  and  probably  act  favorably  besides  in  stimulating  and  regulating 
the  peripheral  circulation.  Additions  of  pine-needle  extract  (1/4  lb.  for  a 
full,  a  few  tablespoonfuls  for  a  partial,  bath)  will  often  increase  the  agreeable 
effect.  Baths  may  be  taken  every  day,  or  later  2  to  3  times  a  week;  the 
patientshould  stay  in  at  first  15  minutes,  afterwards  up  to  an  hour.  Pro- 
tracted baths  may  possibly  produce  an  enfeebling  effect  upon  the  general 
condition,  which  should,  of  course,  be  avoided.  For  insomnia,  the  baths 
will  be  most  effective  if  taken  in  the  late  afternoon. 

No  indication  will  usually  be  found  for  the  use  of  hot- water  baths  (ioo°  F.) 
and  other  general  hot  procedures  which  are,  as  a  rule,  more  fatiguing.  If  a 
cold  seems  to  have  produced  the  palsy — a  rather  rare  occurrence,  by  the  way 
— diaphoretic  treatment  may  be  tried  at  the  outset.  In  cases  of  poisoning, 
too,  especially  those  due  to  certain  metals,  one  may  try  to  help  along  the 
treatment  by  occasional  sweatbaths  and  packs.  Concerning  the  practical 
application  hydrotherapeutic  text-books  should  be  consulted. 

Local  bog,  peat  or  mud  packings  (1220  F.,  duration  from  1/2  to  2  hours) 
most  likely  affect  convalescence  advantageously  during  the  regenerative 
stage;  at  any  rate  they  will  have  a  favorable  effect  upon  symptoms  of  sensory 
irritation  that  still  exist. 

Douches  to  produce  irritation  in  sensory  nerves,  in  disturbances  of  sensi- 
bility, may  be  employed  as  well  as  other  means  which  will  be  considered 
somewhat  later. 

Electro-therapeutics  is  of  much  importance.  It  is  true  that  some  per- 
ipheral paralyses  recover  without  electric  treatment,  but  the  omission  of  an 
indicated  electric  treatment  in  serious  cases  is  always  a  grave  error  in  judg- 
ment. Aside  from  certain  surgical  procedures  and  some  necessary  measures 
of  general  hygiene,  all  other  methods  rather  than  the  electro-therapeutic 
could  be  omitted.  It  is,  however,  not  merely  a  question  of  employing 
"electricity"  but  of  employing  it  skilfully  and  by  the  proper  method.  One 
who  can  not  fulfill  this  demand  should,  as  a  matter  of  principle,  abstain  from 
personally  treating  peripheral  nerve  diseases.  This  may  even  today  be  said 
with  a  full  recognition  of  the  fact  that  the  critical,  even  the  sceptical  reaction 
against  the  former  overrating  of  electro-therapeutics  is  justified.  Of  the 
many  methods  recommended  for  our  purpose,  we  shall  especially  emphasize 
the  following,  approved  by  long  experience.  In  the  present  state  of  our  skill 
and  knowledge  they  will  ordinarily  suffice. 

In  case  of  severe  pains  in  the  early  stage,  stabile  anodal  galvanization,  a 
classic  method  of  treating  neuralgia,  which  will  be  described  later  on,  may  be 


102  DISEASES  OF  THE  PERIPHERAL  NERVES 

used.     The  anode  is  applied  at  the  point  of  greatest  tenderness  or  in  more 
serious  local  lesion  severing  the  nerve,  in  the  region  of  the  central  stump. 

The  method  just  mentioned  is  a  symptomatic  one,  to  be  tried  occasionally, 
while  to  the  following  two  considerable  influence  on  convalescence  and  the 
prospect  of  recovery  is  justly  ascribed. 

The  stabile  cathodal  galvanization  is  applied  when  the  nerve  trunk  has 
suffered  a  lesion,  which  may  be  definitely  localized,  particularly  in  pressure 
paralysis,  but  in  other  traumatic  affections  also.  The  cathode  (electrode 
of  about  20  qcm.)  is  placed  over  the  point  of  lesion,  the  inactive  (dispersing) 
anode  (about  100  qcm.)  on  the  breast  or  back.  The  current  is  increased 
and  diminished  gradually.  Current  strength:  5-6  (4-8)  milliamperes. 
Duration  of  treatment:  5-6-10  minutes.  Seances  to  be  repeated  daily,  at 
least  every  other  day.  Treatment  should  be  begun  a  few  days  after  the 
paralysis  has  set  in.  Reliable  experience  proves  that  it  is  apt  to  hasten  the 
convalescence  (E.  Remak).  Instead  of  leaving  both  electrodes  stationary, 
the  active  electrode,  the  cathode,  may  be  moved  up  and  down  the  course  of 
the  nerve  trunk  to  be  treated,  without,  however,  removing  it  from  the  skin. 

The  actual  galvanic  stimulation  of  the  diseased  region  is  the  most 
important  treatment  in  the  later  stages  of  the  paralysis,  and  may  sometimes 
be  combined  with  the  preceding  method,  but  should  never  be  applied 
before  the  third  week.  The  endeavor  is  to  produce  muscular  contractions 
through  direct  stimulation  of  single  muscles.  Button-shaped  electrodes, 
whose  surface  is  about  10  qcm.  and,  if  desired,  having  a  current  interrupter 
attachment,  are  used  for  this  purpose.  Generally  speaking,  the  muscle  is 
stimulated  with  a  current  of  just  sufficient  strength,  afterwards  with  a 
somewhat  stronger  current,  applying  the  cathode  as  the  irritation  electrode, 
or  in  cases  of  D.  R.  when  the  anode  excites  greater  contractions,  applying 
it  preferably.  Stimulation  is  produced  by  abrupt  closing  of  the  current 
by  the  interrupter,  or  by  moving  the  electrode  over  the  muscle  to  be  stimu- 
lated. In  cases  of  considerable  diminution  of  the  irritability,  alternating 
currents  with  attached  electrodes,  so-called  voltaic  alternating  currents, 
are  employed.  It  is  best  to  let  the  muscles  contract  one  after  the  other, 
and  then  to  begin  again  with  the  first.  In  this  way  each  muscle  of  the 
paralyzed  area  should  be  stimulated  at  first,  say,  10  times,  afterwards  from 
20  to  40  times.  Cylindrical  electrodes  are  also  in  use  with  which  the 
diseased  area  is  traversed  in  a  centripetal  direction.  The  inactive  large 
electrode  is  again  placed  on  the  breast  or  back.  Some  recommend  that  it 
be  placed  on  a  proximate  part  of  the  member  to  be  treated,  or  on  the  nerve 
trunk  whose  muscle  area  is  to  be  treated.  If  the  irritability  is  not  too 
greatly  decreased,  some  stimulations  of  the  nerve  trunk  may  be  added  to  the 
galvanization  of  the  muscles.  Daily  sittings  should  be  held  so  far  as 
possible. 

This  method  may  or  must  be  modified  in  cases  where  great  sensory 


THERAPEUTICS  OF  LOCAL  NEURITIS  103 

irritability,  hyperalgesia,  to  the  electric  current  exists.  All  painful  fluctua- 
tions in  the  current  are  to  be  avoided  by  a  very  gradual  increase  of  the 
current,  after  the  electrode  has  been  attached,  and  moving  the  active  elec- 
trode up  and  down  over  the  area  to  be  treated,  without,  however,  removing 
it  from  the  skin.  The  electrode  is  taken  off  only  at  the  termination  of  the 
treatment  after  the  current  has  been  gradually  diminished. 

Further,  another  method  which  does  not  favorably  compare  with  the 
above  and  which  is  based  on  the  so-called  refreshing  influence  of  the  flow 
of  the  galvanic  current  in  penetrating  the  muscle,  may  be  applied.  Two 
nearly  similar,  medium  sized,  flat  electrodes  are  applied  in  such  a  way  on 
suitable  places  of  the  paralyzed  musculature  that  after  closing  the  current 
it  will  penetrate  the  muscles ;  the  anode  on  the  more  tender  parts  of  the  skin. 
The  current  of  4  to  6  milliamperes  is  gradually  increased  and  diminished. 
The  duration  of  the  seance,  during  which  the  position  of  the  electrodes  may 
be  changed  by  sliding  them  over  the  skin,  should  vary  from  5  to  10  minutes. 

Just  how  these  methods  influence  recovery  in  paralyses,  is  still  prob- 
lematic; but  clinical  experience  has  clearly  shown  that  they  prevent,  to  a 
certain  extent,  muscular  atrophy  and  that  they  promote  recovery.  Some 
experimental  investigations  also  give  support  to  this  statement. 

The  faradic  current  may  be  used  in  those  cases  of  paralysis  in  which 
the  irritability  has  decreased  but  little  and  medium  faradic  currents  suffice 
to  cause  contraction  of  the  muscles.  They  should  be  stimulated  by  stroking 
or  rolling  of  the  musculature,  or  better  still  by  sudden  closing  of  the  current. 
Energetic  prolonged  tetanization  of  the  muscles  can  not  be  advised.  How- 
ever in  motor  paralysis  we  decidedly  prefer  the  galvanic  treatment.  Gal- 
vano-faradization  is  recommended  by  a  number  of  authorities.  Both 
currents  are  brought  simultaneously  to  the  electrodes,  and  the  currents  are 
graduated  in  such  a  way,  that  each  current  just  suffices  by  itself  to  produce 
contractions.  It  is  always  presupposed,  of  course,  that  the  faradic  irritabil- 
ity of  the  parts  concerned  has  not  suffered  severely.  The  faradic  current 
is  usually  contra-indicated  in  motor  and  serious  sensory  conditions  of 
irritation.  In  the  first  stage  of  paralysis  in  which  genuine  irritants  are  to 
be  avoided,  the  faradic  current  should  not  be  employed  under  any  conditions. 

The  faradic  current  is  particularly  to  be  recommended  for  the  treatment 
of  anaesthesia,  if  there  is  no  contra-indication  for  other  reasons.  A  dry, 
brush-shaped  electrode  is  used,  with  which  the  anaesthetic  area  is  stroked 
or,  moving  it  from  place  to  place,  tapped  with  a  current  just  strong  enough 
to  excite  the  sensibility.  By  the  use  of  a  cylindrical  or  movable  button 
electrode  in  galvano-faradization  it  is  possible  to  do  justice  at  one  and  the 
same  time  to  the  indication  of  the  muscle  irritability  and  the  irritation  in 
sensibility. 

The  electric  treatment  should  generally  be  continued  until  convalescence 
is  established.     Treatment  of  a  few  weeks  in  grave  paralysis  will  not  be  effec- 


104  DISEASES  OF  THE  PERIPHERAL  NERVES 

tive.  It  will  be  clearly  seen  in  many  cases  that  in  every  stage  of  the  conva- 
lescence electric  treatment  acts  beneficially,  while  the  omission  of  it  will 
retard  the  progress  of  recovery.  Sometimes,  in  inveterate  cases,  improvement 
sets  in  only  after  the  electric  treatment  has  been  begun.  The  ameliorating 
effect  which  it  frequently  and  directly  produces  on  function,  is  especially 
adapted  to  animate  the  spirits  of  the  patient  with  all  its  consequences. 
Experience  proves  that  patients  hardly  follow  as  faithfully  any  other  method 
for  such  a  long  time  as  they  will  electro-therapeutics,  even  if  the  progress  of 
recovery  be  but  slow. 

This  treatment  should  be  tried  even  in  quite  inveterate  cases.  As  long 
as  the  musculature  is  still  capable  of  being  electrically  irritated  and  the  nerv e 
conductivity  is  not  wholly  destroyed,  even  if  only  a  prospect  for  the  regenera- 
tion of  the  nerve  exists,  there  is  still  hope  for  success. 

In  the  stage  in  which  absolute  rest  does  not  seem  necessary  any  longer, 
massage  materially  assists  electric  treatment  of  the  muscles.  Exact  pro- 
cedure based  upon  anatomy  and  performed  best  according  to  Hoffa's  ex- 
cellent instructions1  is  indispensable. 

Besides  efSeurage,  gentle,  later  vigorous  kneading  and  careful  percussion 
may  be  instituted;  the  more  energetic  manipulations  should  be  used  only  in 
the  stage  of  convalescence.  Even  if  massage  of  the  muscles  may  in  a  few 
exceptional  cases  be  entrusted  to  a  specially  trustworthy  unprofessional 
masseur,  massage  of  the  nerve  trunk  should  be  attempted  only  by  an  experi- 
enced professional  hand  and  then  only  in  a  few  exceptional  cases.  The  very 
great  liability  of  injuring  this  delicate  structure  should  always  be  kept  in 
mind. 

Medical  gymnastics  in  its  widest  sense,  is  absolutely  necessary  in  a  cure 
of  peripheral  motor  paralysis.  As  soon  as  mobility  returns,  each  injured 
muscle  should  be  exercised  systematically.  One  should  never  depend  on  the 
patient's  using  his  muscles  of  his  own  volition  because  he  fails  to  do  so  only 
too  often,  or  if  he  really  uses  them,  he  does  so  usually  to  an  insufficient  degree. 

But  even  in  the  stage  of  complete  paralysis,  as  early  as  the  fundamental 
principles  already  laid  down  will  permit,  gymnastics,  at  first  only  carefully 
executed  passive  movements,  should  be  instituted.  By  this  means  the  devel- 
opment of  contractures  is  prevented.  This  danger  should  be  thought  of  in 
the  beginning  also,  particularly  in  reference  to  the  position  of  the  diseased 
parts.  In  a  peroneus  paralysis  a  pes  equinus  position  is  not  to  be  tolerated; 
in  a  radialis  paralysis  the  hand  is  not  to  be  left  flexed.  Proper  support  and 
bandages  should  keep  the  member  in  a  medial  position.  Above  all,  passive 
movements  promote  the  return  of  active  motility.  The  patient  should  from 
the  beginning  endeavor  actively  to  assist  the  passive  motion.  The  moving 
hand  perceives  immediately  when  these  endeavors  begin  to  be  successful. 
Then  the  passiye  motion  is  replaced  by  passive-active  gymnastics.     The  dis- 

1  His  "technic  of  massage"  (Stuttgart,  1903)  is,  in  its  technical  suggestions,  authoritative. 


THERAPEUTICS  OF  LOCAL  NEURITIS  105 

eased  member  requires  aid  for  some  time  yet,  if  it  is  to  utilize  the  existing 
minimum  of  strength  gymnastically.  This  support  is  rendered  by  the  hand 
of  the  physician  or  his  assistant,  who  have  so  far  moved  the  member  only 
passively.  In  many  cases  an  unaffected  hand  of  the  patient  may  render  the 
assistance,  in  suitable  cases,  by  means  of  a  skilfully  attached  strap.  Thus 
if  a  part  is  to  be  dorsally  flexed  a  strap  may  be  attached  to  it  and  worked 
by  the  patient.  An  excellent  means  of  aiding  the  first  attempts  in  active 
gymnastics  is  afforded  by  warm  baths.  The  laws  of  physics  enable  the 
patient  to  execute  motions  independently  in  the  bath  which  he  is  unable  to 
make  otherwise  (kinetotherapeutic  baths)  and  this  is  a  particularly  important 
point  indicating  the  bath  treatment  (Goldscheider) . 

If  apparatus  for  so-called  semi-active  gymnastics  is  at  one's  disposal, 
apparatus  which  mechanically  assists  the  performance  of  the  patient  and 
enables  him  to  make  movements  with  the  expenditure  of  but  little  strength, 
like  the  Krukenberg  pendulum  apparatus  and  similar  contrivances,  it  may 
be  used. 

With  further  progress  toward  recovery  the  real  movement  exercises  and 
finally  the  various  forms  of  resistance  gymnastics  are  reached. 

As  a  general  rule  for  gymnastic  therapy  it  may  be  stated,  that  here  as 
everywhere,  the  patients  should  exercise  frequently,  but  not  long  at  a  time; 
that  they  should  never  be  tired  out  or  too  much  exhausted  by  it. 

Surgical  treatment,  in  many  cases  the  conditio  sine  qua  non  of  recovery, 
is  discussed  separately  in  this  work.  We  shall  therefore  simply  suggest  here 
the  general  possibility  of  its  application. 

When  a  nerve  is  functionally  injured  by  surrounding  scar  tissue,  by  pres- 
sure of  masses  of  callus,  or,  in  a  similar  manner,  neurolysis,  notwithstanding 
the  fact  that  in  some  cases  a  spontaneous  recovery  may  obtain,  should,  as  a 
rule,  be  used  promptly  as  it  sometimes  restores  the  nerve  conductivity  directly. 
A  completely  divided  nerve  should  be  sewn,  if  possible,  primarily,  as 
restoration  without  the  reunion  of  the  nerve-ends,  despite  some  sporadic, 
anomalous  cases,  not  as  yet  explained,  can  not  be  expected.  If  any  doubt 
exists  as  to  the  necessity  of  a  suture,  it  is  in  most  cases  advisable  to  look  for 
the  nerve  operatively,  and  to  decide  then  whether  or  not  to  suture  it.  In 
inveterate  cases,  where  the  course  has  demonstrated  that  an  amelioration  is 
not  to  be  expected  without  surgical  intervention,  the  different  methods  of 
even  secondary  nerve  suture  promise  success.  If  this  is  impracticable  the 
advisability  of  nerve  grafting  will  have  to  be  considered. 

Of  course  in  all  these  cases,  with  but  few  exceptions  which  are  difficult  to 
explain,  a  long  time  will  elapse  before  functional  restoration  takes  place. 

If  the  morbid  process  has  entirely  ceased,  and  it  is  quite  obvious  that  a 
change,  either  for  better  or  for  worse,  will  not  set  in,  and  if  the  procedures  for 
the  restoration  of  innervation  are  no  longer  a  point  of  issue,  orthopedics  are 
in  order.     Attempts  should  be  made  to  give  the  paralyzed  member  support 


106  DISEASES  OF  THE  PERIPHERAL  NERVES 

by  means  of  immobilization  apparatus,  by  arthrodesis,  by  operative  short- 
ening of  relaxed  tendons.  Contractures  are  relaxed  by  tenotomy  or  by 
plastic  operations  on  tendons;  unimpaired  muscles,  by  transplantation  of 
tendons,  are  made  to  serve  as  substitutes  for  muscles  whose  functions  have 
been  lost. 

In  many  cases  such  measures  will  produce  extraordinary  results.  But 
one  must  not  expect  too  much  from  orthopedic  operations.  If  the  patient  is 
used  to  an  old  defect,  and  endures  it  without  considerable  impairment  of 
his  well-being  and  his  capabilities,  it  would  mostly  be  well  to  omit  surgical 
operations,  the  result  of  which  is  never  quite  certain. 

Finally  let  it  be  particularly  emphasized  that  besides  operative  procedures 
the  other  methods  of  treatment  should  never  be  neglected. 

PARALYSIS  OF  INDIVIDUAL  PERIPHERAL  NERVES 

Although  in  the  following  the  clinical  picture  of  diseases  of  individual 
peripheral  nerves  is  to  be  discussed,  it  will  not  be  done  with  exclusive  re- 
gard for  the  clinical  group  of  single  diseases.  We  shall  keep  in  mind  that  a 
great  part  of  the  paralyses  to  be  treated  here,  appear  as  partial  symptoms  also 
in  polyneuritis. 

A.  The  Cranial  Nerves 

i.  The  Olfactory  Nerve 

It  is  obvious  that  the  olfactory  nerve  may,  in  a  number  of  intra-cranial 
diseases,  be  affected  unilaterally  or  bilaterally.     In  tabes  it  may  atrophy. 

Independent  isolated  affections  of  the  olfactory  nerve  are  rare.  Only 
one  type  is  of  some  practical  interest.  After  trauma  of  the  skull,  anosmia, 
which  can  be  ascribed  to  the  mechanical  destruction  of  the  olfactory  filaments, 
may  occur.  The  loss  of  the  sense  of  smell  may  mean  an  impairment  in  the 
ability  to  make  a  living,  especially  in  those  occupations  in  which  the  tasting 
and  "smelling"  of  food  or  drink  is  essential,  and  in  others  in  which  the  organ 
of  smell  is  necessary  to  protect  one's  self  from  dangers,  such  as  poisonous 
gases  (chemical  factories,  etc.). 

Destruction  of  the  olfactory  nerves  results  in  loss  of  smell.  At  the  same 
time,  however,  there  is  involved  great  impairment  of  the  ability  for  what  is 
colloquially  termed  "tasting."  The  ability  to  taste  is  in  organic  anosmia 
restricted  to  the  elementary  taste  qualities  (sweet,  sour,  etc.).  On  this  the 
differential  diagnosis  in  regard  to  hysteric  anosmia  may  be  based.  One 
affected  with  organic  anosmia,  despite  the  preservation  of  the  organs  of 
taste,  lacks  the  ability  to  distinguish  all  those  articles  by  the  "taste,"  for 
whose  recognition,  unknown  to  most  people,  the  co-operation  of  the  sense  of 
smell  is  necessary.  If  this  ability  is  preserved  in  spite  of  anosmia,  it 
points  unquestionably  against  an  organic  foundation. 


THE  OLFACTORY  AXD  OPTIC  NERVES  107 

It  must  be  remembered  that  by  far  the  most  frequent  cause  of  anosmia  is 
nasal  disease,  and  that  it  must  be  carefully  excluded  before  nervous  anosmia 
is  diagnosed.  In  obstruction  of  the  posterior  nares  the  sense  of  smell,  in 
subserving  taste,  is  much  diminished.  Patients  complain  about  disturbances 
of  taste,  even  though  the  sensitiveness  of  the  organ  of  taste  for  the  elemen- 
tary qualities  is  normal  and  though  odors  are  recognized  by  the  nose 
(gustatory  anosmia). 

Congenital  defects  of  the  olfactory  nerve  also  occur.  The  fundamental 
factors  of  senile  anosmia,  and  of  that  which  sometimes  remains  after  nasal 
disease,  are  still  unknown. 

2.  The  Optic  Nerve 

The  diseases  of  the  optic  nerve  of  interest  to  the  neurologist  are  those 
which  occur  most  often  as  part  of  the  symptomatology  of  certain  cerebral  and 
spinal  diseases.  Isolated  diseases  of  the  optic  nerve,  as  a  rule,  fall  to  the  lot 
of  the  eye  specialist.  Nevertheless,  especially  in  the  interest  of  differential 
diagnosis,  a  survey  of  the  general  field,  which  we  shall  give  in  the  following 
paragraphs,  is  necessary  to  the  neurologist. 

The  optic  nerve,  in  the  morphologic  embryological  sense,  is  not  a  per- 
ipheral nerve,  but  a  projected  portion  of  the  brain.  Of  especial  significance 
for  an  understanding  of  its  diseases  is  the  fact  that  the  fibres  serving  to  give 
the  keenest  central  vision,  belonging  to  the  yellow  spot  (macula  lutea),  form 
in  the  nerve  trunk  a  large  separate  bundle.  This  papillo-macular  bundle 
first  occupies,  near  the  bulbus,  a  peripheral  position  corresponding  to  the 
temporal  border  of  the  disc,  while  further  on  it  runs  in  the  axial  part  of  the 
transverse  section. 

The  chief  symptom  of  disease  of  the  optic  nerve  is  clearly  disturbance 
of  vision  of  various  forms,  which,  however,  differ  from  those  occurring  in 
affections  of  the  chiasm,  the  optic  tract,  and  the  more  central  parts  of 
the  visual  tract,  which  are  distinguished  by  their  peculiar  hemianopic 
character,  and  which  disease  of  the  optic  nerve  does  not  produce.  (Con- 
cerning hemianopsia,  cf.  the  chapter  on  brain  diseases.) 

In  serious  disease  of  an  optic  nerve  the  eye  concerned,  almost  without 
exception,  does  not  react  to  light  by  contraction  of  the  pupil.  Of  more 
theoretical  interest  than  practical  importance  is  the  fact  that  in  very  rare 
cases,  where  total  blindness  resulted  from  atrophy  of  the  optic  nerve,  the 
optic  nerve  concerned  was  said  to  have  transmitted  pupillary  light  reflexes. 
According  to  this  it  would  seem  that  special  fibres  subserving  the  reaction  to 
light  could  withstand,  on  occasion,  the  morbid  processes  longer  than  the 
tracts  destined  for  vision,  though  even  in  these  cases  the  pupillary  light 
reaction  never  was  totally  undisturbed. 

In  most  diseases  of  the  optic  nerve  ophthalmoscopic  changes  of  a  charac- 
teristic nature  are  known  to  take  place  in  the  disc  of  the  optic  nerve. 


108  DISEASES' OF  THE  PERIPHERAL  NERVES 

For  various  reasons  a  classification  of  optic-nerve  diseases  is  very  difficult. 
Concerning  the  pathological  anatomy  there  is  no  unanimity  among  the  most 
experienced  investigators,  and  still  less  clear  in  many  respects  is  the  patho- 
genesis. The  same  visible  changes  in  the  fundus  are  occasioned  by  various 
conditions,  and,  on  the  other  hand,  the  same  injury  may  occasion  quite 
different  anatomic  and  ophthalmoscopic  pictures.  Finally,  the  ophthal- 
moscopic findings  change  considerably  in  various  stages  of  the  same  process. 

The  following  classification  will  suffice  for  all  practical  purposes,  though 
it  is  necessarily  lacking  in  unity. 

{a)  Primary  {Idiopathic)  Atrophy 

Idiopathic  atrophy  is  diagnostically  a  very  important  symptom,  because 
it  often  appears  very  early,  of  tabetic  and  paralytic  diseases.  It  will  be 
treated  more  minutely  in  its  relation  to  those  diseases  in  the  proper  place. 
Clinically  it  is  characterized,  besides  the  ophthalmoscopic  findings  of  an 
always  bilateral,  simple  diffuse  atrophy,  by  a  more  or  less  severe,  usually 
irregular,  but  concentric  contraction  of  the  field  of  vision.  The  sense  of 
color  suffers  likewise  (at  first  for  red  and  green),  and  so  does  central  vision. 
The  fibres  of  the  optic  nerve  disappear,  and  likewise  very  early  their  cells 
of  origin  in  the  retina.  Simultaneously  with  the  atrophy,  there  gradually 
develops  a  disturbance  in  sight,  which  usually  progresses  till  total  blindness 
results. 

The  atrophy  of  the  optic  nerve  in  glaucoma,  which  is  of  purely  oculistic 
interest,  may  always  be  distinguished  definitely  from  tabo-paralytic  atrophy 
by  ophthalmoscopic  examination. 

The  atrophy  of  the  optic  nerve  in  the  Tay-Sachs  amaurotic  family  idiocy 
is  probably  the  result  of  a  serious  disease  of  the  retina.  The  ophthalmo- 
scopic picture,  the  circumstances  and  the  symptoms  accompanying  its 
appearance  are  so  characteristic  that  it  never  presents  any  difficulty  in 
differential  diagnosis. 

The  secondary  atrophy  after  papillitis  (neuritic  atrophy)  will  be  discussed 
in  the  following  paragraphs.  It,  too,  is  differentiated  from  "simple" 
atrophy  by  the  peculiar  ophthalmoscopic  findings,  which  will  in  most 
cases  enable  its  recognition  and  differentiation,  especially  in  the  early 
stages  in  which  the  differential  diagnosis  of  the  affection  of  the  optic 
nerve  is  of  prime  importance  for  a  decision  as  to  the  fundamental  morbid 
condition. 

The  primary  tabetic  and  paralytic  atrophy  may  most  easily  be  con- 
founded with  certain  secondary  simple  atrophies,  which  appear  most  fre- 
quently in  connection  with  retrobulbar  processes  of  the  most  varied  nature. 
They  are  treated  of  under  (c)  and  (d),  where  reference  is  made  to  the  peculiar 
characteristics  of  the  different  forms  in  this  group. 


DISORDERS  OF  THE  OPTIC  NERVE  109 

(b)  The  Forms  Occurring  under  the  Aspect  of  Papillitis,  Especially  Choked 
Disc  and  Primary  N euro-papillitis ,  Neuritis  Optica  Intraocularis 

In  its  incipient  stage  the  prognosis  of  a  papillitis  may  present  considerable 
difficulties.  When  the  physiological  tortuosity  of  the  vessels  of  the  retina 
is  strongly  marked,  and  the  disc  is  hyperemic,  as  is  habitually  the  case  in 
hypermetropia,  it  is  really  a  case  of  pseudo-neuritis  optica.  The  concentric 
wrinkling  of  the  retina  in  the  neighborhood  of  the  disc,  which  is  occasionally 
observed  in  the  beginning  of  true  papillitis,  may  make  a  differential  diagnosis 
possible. 

1.  Choked  disc  occurs  as  a  result  of  space  contracting  intra-cranial 
processes,  or,  to  be  more  precise,  of  such  conditions  as  prevent  the  free  flow 
of  the  venous  and  lymphatic  circulations  in  the  cavity  of  the  skull.  This 
causes  trouble  in  the  area  of  one  or  usually  of  both  optic  nerves,  which  is 
the  immediate  cause  of  the  development  of  choked  disc.  Disease  processes 
in  the  orbit,  too,  may  in  rare  cases  lead  to  analogous,  though,  of  course, 
unilateral,  alterations.  Choked  disc  will  be  treated  in  more  detail  with  the 
different  intra-cranial  diseases  especially  with  brain  tumor.  From  choked 
disc  it  is  not  always  easy  by  means  of  an  ophthalmoscope  clearly  to  dis- 
tinguish the  next  affection. 

2.  Primary  Neuritis  Optica  Intraocularis. — The  histological  and 
pathogenic  comprehension  of  choked  disc  in  its  more  important  manifesta- 
tions is  not  yet  very  definite,  and  this  holds  true,  perhaps  even  to  a  higher 
degree,  in  the  case  of  neuritis  optica.  In  the  latter,  it  is  usually  a  case  of 
inflammatory  processes,  while  choked  disc,  at  least  in  the  early  stage, 
shows  only  congestive  symptoms,  especially  edema.  As  a  primary  inde- 
pendent disease,  neuritis  optica  has  been  traced  back  to  all  sorts  of  sources, 
frequently  on  very  questionable  grounds.  Its  occurrence  in  connection 
with  various  infectious  diseases,  intoxications  (lead),  and  with  intra-cranial 
infectious  inflammations,  is  certain.  It  is  supposed  that  it  may  be  directly 
produced  by  anemic  conditions  and  severe  hemorrhage,  Worthy  of  mention 
is  a  hereditary  form,  usually  appearing  at  puberty  in  males,  and  being 
transmitted  through  the  female  line. 

3.  Papillitis  in  inflammation  of  the  retina  (albuminuric  retinitis  et  al.), 
changes  of  the  disc  in  thrombosis  of  the  central  vein  and  in  embolism  of  the 
central  artery,  have  a  decided  differential  diagnostic  interest  for  the 
neurologist. 

All  forms  of  papillitis  are  accompanied  by  more  or  less  serious  dis- 
turbances in  vision.  These  may  recover  or  result  in  so-called  secondary 
neuritic  atrophy,  which  ophthalmoscopically  is  characterized  by  certain 
peculiarities. 

In  choked  disc,  vision  more  frequently  remains  normal  for  a  considerable 
time. 


no  DISEASES  OF  THE  PERIPHERAL  NERVES 

(c)  Diseases  Beginning  in  the  Retrobulbar  Region  (Retrobulbar  Neuritis) 

In  cases  of  this  group,  the  disorder  begins  with  a  destructive  influence 
acting  upon  that  section  of  the  optic  nerve  which  is  retrobulbar.  Hence 
the  first  clinical  symptoms  are  disturbances  in  vision,  sometimes  total 
blindness,  sometimes  peripheral  defects  in  the  field  of  vision,  sometimes 
disturbance  of  central  vision  without  impairment  of  the  peripheral  parts  of 
the  field  of  vision,  so-called  central  scotoma,  which  indicate  an  isolated  dis- 
ease of  the  papillo-macular  bundle,  for  which,  according  to  the  latest  investi- 
gations, an  anatomic  predisposition  to  peculiarities  in  the  supply  of  vessels 
of  this  area,  is  apparently  to  be  held  responsible  (Birch  Hirschfeld). 

According  to  the  place,  nature  and  mode  of  attack  of  the  influencing 
injuries,  ophthalmoscopic  changes  occur  in  a  few  cases  relatively  early,  as 
neuritis  optica,  which  may  be  caused  by  a  descending  inflammation,  or 
choked  disc.  In  such  cases  the  disorder  may  terminate  in  atrophy  of  the 
disc. 

In  many  cases,  however,  the  visible  changes  of  the  papilla  of  the  optic 
nerve  occur  only  later,  when,  according  to  the  extent  and  gravity  of  the  dis- 
ease, it  appears  as  a  more  or  less  marked,  usually  only  partial,  especially  often 
temporary,  simple  atrophic  discoloration. 

Retrobulbar  optic-nerve  disease  is  as  a  rule  unilateral. 

From  the  facts  stated,  the  necessary  general  basis  for  differential  diagnosis 
becomes  apparent. 

Not  considering  the  rare  focal  diseases,  such  inflammatory  processes  as 
occur  in  myelitis  and  meningitis,  and  the  metastatic  abscesses  of  the  optic 
nerve  which  are  sometimes  observed,  the  following  diseases  must  be  con- 
sidered chiefly. 

(i)  Diseases  due  to  pressure.  The  effect  of  long  continued  pressure  on 
the  nerve  trunk,  occurring  in  tumors  in  the  immediate  vicinity,  in  aneurisms 
of  the  internal  carotid  or  ophthalmic  artery,  in  old  age  perhaps  even  in 
simple  arterio-sclerosis  of  these  vessels,  usually  is  simple  atrophy. 

Constant  pressure  on  the  chiasm  (tumors  of  the  hypophysis)  or  the  optic 
tract  may  also  lead  to  descending  atrophy.  Sight  disturbances  in  these  cases 
generally  are  hemianopic;  many  diseases  of  the  chiasm  even  lead  to  total 
blindnesss. 

In  cases  of  very  slowly  increasing  pressure,  it  may  occur  that  disturbances 
in  sight  will  not  be  noticed  until  visible  atrophy  has  set  in,  which  is  often 
easily  confounded  with  simple  atrophy. 

(2)  The  tumors  of  the  optic  nerve  itself,  of  which  many  primary  and 
metastatic  forms  occur.     As  a  rule  they  lead  to  papillitic  processes. 

(3)  Other  orbital  diseases  of  all  kinds  may  encroach  upon  and  affect  the 
optic  nerve. 

(4)  The  affections  of  the  optic  nerve  in  diseases  of  the  posterior  accessory 


CAUSES  OF  OPTIC  NEURITIS,  TOXIC  AMBLYOPIA  m 

cavities  of  the  nose,  of  the  sphenoid  and  ethmoid  cavities,  which  stand  in 
close  anatomic  relation  to  the  optic  trunk. 

(5)  Malformations  .of  the  skull,  especially  the  so-called  tower-shaped 
head,  frequently,  even  in  infancy,  lead  to  disease  of  the  optic  nerve. 

(6)  Injuries  of  the  optic  nerve  caused  directly  by  gunshot  or  knife  wounds, 
and  more  frequently  by  fracture  of  the  skull,  show  naturally  all  degrees  of 
lesion  up  to  the  complete  rupture  of  the  nerve.  In  the  beginning  the  disc  may 
be  hyperasmic  or  edematous;  in  ruptures  of  the  central  vessels  it  presents  the 
appearance  of  grave  anaemia.  Very  often  the  ophthalmoscopical  picture  is 
at  first  quite  normal.  The  outcome  is  always  an  atrophy,  usually  simple, 
corresponding  in  degree  to  the  injury.  The  atrophy  of  the  optic  nerve 
which  is  sometimes  observed  in  small  children  after  delivery  by  means  of 
forceps,  may  also  be  included  here. 

In  some  rare  instances,  after  cranial  trauma,  bilateral  atrophy  of  the  optic 
nerves  has  gradually  developed,  which  clinically  has  usually  taken  the  same 
course  as  primary  tabo-paralytic  atrophy. 

(7)  Diseases  of  the  optic  nerve  in  multiple  sclerosis. 

Multiple  sclerosis  very  frequently  leads  to  the  development  of  focal  lesions 
in  one  or  both  optic  nerves.  The  peculiarity  of  this  disease  to  leave  the  axon 
intact  for  a  comparatively  long  time,  explains  some  singularities  in  the  clinical 
picture  of  this  disease  of  the  optic  nerve.  Disturbances  of  vision  may  be 
entirely  wanting,  even  in  cases  in  which  an  atrophy  ophthalmoscopically 
proven,  most  commonly  with  its  characteristic  picture  of  temporal  pallor, 
exists.  In  the  course  of  the  further  development  of  the  malady,  slight  dis- 
turbances in  vision  may  still  set  in.  They  rarely,  if  ever,  develop  to  such  a 
degree   as  to  result  in  marked  diminution  of  vision,  let  alone  blindness. 

Not  infrequently  acute  disturbances  in  vision  are  observed  in  the  early 
stage  of  the  disease,  mostly  of  one  eye,  as  a  rule  in  the  form  of  central  sco- 
toma, occasionally  with  transient  papillitic  changes,  and  in  exceptional  cases 
even  choked  disc.  These  sight  disturbances  are  generally  more  or  less  com- 
pletely recovered  from.  They  are  probably  coincident  with  the  early  stage 
of  a  sclerotic  focus  in  the  optic  nerve. 

(d)    Toxic  Amblyopias  and  a  Few  Related  Forms  of  Acute  Blindness 
Originating  Peripherally 

That  many  different  intoxications  may  lead  to  the  development  of  papil- 
litis has  already  been  mentioned.  The  toxic  amblyopias  occupy  a  unique 
position  with  respect  to  these  affections.  Under  this  name  acute  and  chronic 
diseases  are  grouped  together. 

The  toxic  amblyopia  of  chronic  development,  observed  most  frequently 
in  chronic  tobacco  and  alcohol  poisoning,  may  be  considered  as  one  of  the 
retrobulbar  diseases  which  we  have  just  discussed,  though  it  is  as  a  rule 


ii2  DISEASES  OF  THE  PERIPHERAL  NERVES 

bilateral.  Concerning  its  pathological  anatomy  and  pathogenesis,  nothing 
conclusive  is  known,  but  the  clinical  picture  indicates  that  the  papillo-macular 
bundle  is  chiefly  affected.  The  disturbance  in  vision  is  a  characteristic 
scotoma,  first  for  red  and  green,  later  on  for  white,  while  the  field  of  vision 
otherwise  remains  normal.  In  the  later  stages,  a  slight  pallor,  especially  of 
the  temporal  part  of  the  optic  nerve  head  may  set  in.  Mistaking  it  for  in- 
cipient tabetic  atrophy  can  be  easily  avoided  by  observing  carefully  the 
characteristic  functional  disturbances.  The  prognosis  is  relatively  favorable; 
that  of  the  tabetic  atrophy,  unfavorable. 

Bilateral  toxic  amblyopias  and  amauroses  of  peripheral  origin  setting  in 
acutely  occur  in  many  acute  poisonings,  among  which,  those  due  to  quinine,1 
male  fern,  and  methyl  alcohol  should  be  mentioned.  They  have  been  also 
observed  to  result  from  prolonged  use  of  atoxyl.  Very  similar  clinical 
pictures  may  be  produced  by  severe  hemorrhage,  and,  after  employment  of 
X-rays  or  other  rays  of  somewhat  similar  character.  They  are  occasioned 
by  grave,  acute  diseases  of  the  ganglionic  retinal  nerve  cells,  and  often  of  the 
fibres  themselves.  A  partial  restoration  is  possible  but  the  usual  termi- 
nation is  a  more  or  less  complete  atrophy  of  the  optic  nerve. 

What  produces  the  grave  disease  in  the  optic  apparatus  in  these  and  other 
cases  in  which  general  injurious  influences  are  at  work,  is  not  known. 

The  therapeutics  of  the  diseases  of  the  optic  nerves  must  proceed 
according  to  fundamental  principles.  In  cases  of  atrophy,  in  which  amelio- 
ration, or  at  least  a  cessation  of  the  process,  does  not  seem  impossible,  galvanic 
treatment  should  be  tried,  though  the  recent  optimistic  reports  concerning 
the  success  of  such  treatment  need  to  be  more  fully  verified.2  The  anode  is 
placed  upon  the  closed  eyelid,  the  cathode,  which  should  be  larger,  upon 
the  neck.  Strong  currents,  carefully  increased  and  diminished,  and  pro- 
tracted seances  (15  minutes  and  longer)  are  recommended.  Sometimes  long 
continued  treatment  with  strychnine  appears  to  be  useful. 

3.  Nerves  of  the  Eye  Muscles.     Oculomotor,  Trochlear  and 

Abducens  Nerves 

The  palsies  resulting  from  disease  of  the  motor  nerves  of  the  eye,  in  a  great 
number  of  cases  can,  practically  not,  be  distinguished  with  certainty  from  those 
resulting  from  intra-cerebral  injury  of  the  same  peripheral  motor  neuron. 
Even  theoretically,  in  a  large  group  of  cases,  it  can  not  be  authoritatively 
stated  whether  they  may  be  considered  as  peripheral  nerve  disease  or  not. 

Palsies  due  to  progressive  nuclear  (spino-bulbar)  muscular  atrophy, 
cases  of  polioencephalitis  haemorrhagica  superior,  probably  most  cases  of 
congenital  palsies  caused  by  imperfect  development  of  the  nuclei  which 
often  assume  the  character  of  supranuclear  paralysis,  and  finally  the  paraly- 

1  The  minimum  dose  that  has  occasioned  disturbances  in  vision,  is  5  grm.  in  30  hours. 

2  Cf .  Mann,  Zeitschrif t  fur  diatet.  und  physic.    Therapie,  volume  VIIL 


OCULAR  PALSIES  113 

ses  occurring  in  focal  disease  of  the  crusta,  either  injuring  the  nuclei  them- 
selves or  affecting  the  intra-cerebral  root-bundle  sympathetically  (fascicular 
paralyses),  all  these  can  be  traced  back  to  nuclear  or  at  least  to  intra-cere- 
bral injuries  or  diseases. 

In  contrast  to  these  forms,  which  are  mentioned  here  only  for  the  purpose 
of  differential  diagnosis,  stand  others  which  most  certainly  are  peripheral 
nerve  diseases,  i.  e.,  the  large  group  of  basal  and  orbital  paralyses.  Among  the 
diseases  which  cause  paralysis  by  lesion  of  the  nerves,  acute  and  chronic  men- 
ingitis from  whatever  cause,  and  especially  syphilitic  chronic  basal  meningitis, 
must  be  first  mentioned.  Injury  to  the  nerves  caused  by  the  pressure  of  tumors 
must  also  be  considered.  These  tumors  may  be  situated  in  the  meninges 
themselves,  though  more  frequently  those  of  the  base  of  the  skull  (sarcomata), 
and  above  all,  tumors  of  the  brain  are  the  cause  of  the  paralyses  through 
pressure.  It  is  very  important  to  notice,  that  tumors  of  each  brain  region 
may  occasionally  cause  ocular  paralysis.  Tumors  in  the  region  of  the  pos- 
terior cranial  fossae  most  frequently  injure  the  abducens;  those  of  the  middle 
cranial  fossae,  especially  of  the  temporal  lobes,  may  lead  to  grave  and  extensive 
ophthalmoplegia  through  pressure  on  the  nerve  trunks,  which  are  closely 
interwoven  at  the  base  of  the  brain,  as  is  the  case  with  the  tumors  of  the 
frontal  region  when  they  spread  towards  the  superior  orbital  fissure.  In 
the  cases  named,  the  mechanism  of  the  pressure  effect  is  clearly  understood. 
But  sometimes  there  are  cases  where  it  is  very  difficult  to  understand  the 
relation  between  a  brain  tumor  located  at  some  distance  from  the  base,  and 
some  coincident  forms  of  slighter  paralyses  in  the  eye  muscles.  Neoplasms 
very  rarely  directly  involve  the  nerves.  It  is  exceedingly  seldom  that  tumors 
arise  on  the  nerves  themselves  (primary  and  metastatic). 

Besides  morbid  growths,  arterio-sclerotic  changes  in  vessels,  aneurisms 
of  the  internal  carotid  and  other  arteries,  can  injure  the  nerve  trunks  through 
pressure. 

Like  tumors  of  the  temporal  lobes,  abscesses  of  the  same  region  affect  the 
nerves  of  the  base. 

Thrombosis  of  the  cerebral  sinuses,  particularly  of  the  sinus  cavernosus, 
frequently  lead  to  paralysis  of  the  eye  muscles. 

Of  diseases  of  the  orbit,  it  is  mainly  inflammation  and  tumors  or  tumor- 
like structures  that  play  an  aetiological  role. 

Of  the  traumatic  palsies,  those  resulting  from  fracture  of  the  base  of  the 
skull  are  of  the  greatest  interest. 

Trauma  and  syphilis  may  likewise  in  rare  cases  lead  to  paralyses  from 
intra-cerebral  injury  or  disease.  The  trauma  may  cause  hemorrhages  in  the 
nuclear  region,  while  syphilis  is  likely  to  induce  chronic  progressive 
disease  of  the  nuclei. 

Those  cases  that  now  and  then  are  observed  in  connection  with  ophthalmic 
herpes  zoster  are  probably  also  of  neuritic  origin. 


ii4  DISEASES  OF  THE  PERIPHERAL  NERVES 

For  an  exceedingly  large  group  of  paralyses,  as  has  been  said  before,  no 
definite  classification  which  would  serve  for  all  cases  can  be  made,  since  the 
known  pathologic-anatomical  findings  vary,  at  least  enough  to  make  differ- 
ent explanations  possible.  This  is  the  case  with  paralysis  of  the  eye  muscles 
in  tabes  and  paresis,  in  multiple  sclerosis,  and  in  the  cases  connected  with 
infectious  and  toxic  diseases.  Among  the  infectious  diseases,  diphtheria  and 
influenza  stand  in  the  front  rank  (cf .  also  chapter  on  polyneuritis) .  Among 
the  toxic  processes,  sausage  poisoning  (botulismus)  and  lead  poisoning  deserve 
to  be  mentioned.  'The  cases  observed  in  Basedow's  disease  and  diabetes 
most  likely  depend  on  autointoxication.  How  in  all  these  cases  there 
results  a  localized  affection  of  special  eye  muscles  is  entirely  unknown. 
Very  uncertain  is  the  astiological  significance  of  catching  cold  or  being  chilled, 
so  often  affirmed.     In  many  cases  all  discoverable  causes  are  lacking. 

Clinical  Conditions  and  the  Course  of  the  Disease. 

Paralyses  of  the  eye  muscles  appear,  as  is  evident  from  the  aetiological 
discussion,  as  component  symptoms  of  the  most  varied  conditions.  How- 
ever, they  appear  also  as  an  independent  disease. 

In  either  case  the  affection  may  be  restricted  to  one  of  the  cranial  nerves 
or  to  a  nerve  pair.  Isolated  oculomotor  and  abducens  palsies  are  frequent; 
isolated  trochlear  palsies  rare.  The  trochlear  is  more  frequently  affected  in 
combination  with  other  ocular  nerves. 

In  oculomotor  affections,  total  paralysis  and  paresis  affecting  all  branches 
of  the  nerve  must  be  distinguished  from  partial  functional  disturbances, 
restricted  to  a  few  single  muscles  of  its  innervation  area. 

In  total  oculomotor  paralysis  the  mobility  of  the  eye  is  practically  gone. 
As  the  abducens  and  trochlearis  are  functionally  unimpaired,  the  eye  de- 
viates considerably  outward  and  a  trifle  downward.  Every  attempt  to 
innervate  motorially  the  ball  is  apt  to  increase  this  deviation.  The  eye  is 
diverted  still  further  into  the  outer  canthus,  while,  because  the  trochlearis 
still  acts,  the  upper  pole  of  the  vertical  meridian  of  the  cornea  is  at  the  same 
time  turned  slightly  inward.  The  increase  in  deviation  due  to  an  attempt  at 
innervation,  no  matter  how  directed,  must  be  looked  on  as  a  so-called  com- 
pensation movement  and  is  explained  thus :  that  an  innervation  current  flows 
off  into  the  open  channels,  since  its  own  are  closed.  The  upper  eyelid  is 
dropped  and  can  be  raised  only  passively.  The  pupil  is  more  than  medium 
wide,  the  contraction  reactions  are  lacking,  the  accommodation  is  paralyzed. 
Often  there  co-exists  a  slight  degree  of  exophthalmus.  The  eye,  since  its 
muscular  fixedness  is  lacking,  usually  almost  protrudes  from  the  orbit. 

Partial  oculomotor  paralysis  may  affect  each  muscle  separately  or  an 
irregular  group  of  muscles.  To  the  most  frequent  occurrences  of  this  nature 
belongs    isolated    paralysis   of  the  levator  palpebral    (ptosis).     Important 


OCULOMOTOR  PARALYSIS  115 

typical  combinations  are  the  so-called  external  oculomotor  paralyses,  in 
which  only  the  external  branches  of  the  nerve  are  paralyzed,1  and  the  con- 
trasting ophthalmoplegia  interna,  in  which  the  paralysis  is  confined  to  the 
internal  muscles  of  the  eye.  It  is  of  special  importance  to  know  the  condi- 
tions in  convalescence  of  the  latter,  and  of  great  practical  interest,  too.  As  a 
rule,  first  the  palsy  of  the  ciliary  muscle  decreases,  and  the  accommodation 
naturally  returns,  while  the  pupil  does  not  contract  either  to  light  rays  or  by 
convergence.  (A  condition  known  as  pupillary  immobility,  "Pupillenstarre.") 
In  the  further  course  the  convergence  reaction  returns  before  the  light  reac- 
tion, so  that  in  this  stage  it  is  easy  to  confuse  this  condition  with  that  of 
purely  reflex  pupillary  immobility,  which,  diagnostically,  is  so  extremely 
significant.  For  the  differential  diagnosis  it  is  important  first  to  note  that 
ophthalmoplegia  interna  frequently  occurs  unilaterally,  while  the  true 
Argyll-Robertson  phenomenon,  on  the  other  hand,  occurs  unilaterally  only 
in  exceptional  cases.  In  residual  ophthalmoplegia  interna,  the  pupil  as  a  rule 
is  of  an  average  or  slightly  larger  width,  while  the  reflex  pupillary  immobility 
often  combines  with  miosis.  The  most  important  point  is,  that  in  reflex 
pupillary  immobility  the  convergence  reaction  always  remains  absolutely 
normal.  In  the  spurious  form  slight  residua  of  a  disturbance  can  always  be 
found,  even  in  regard  to  the  convergence  reaction. 

Besides  thus  grouping  these  cases  according  to  the  extension  of  the  para- 
lytic symptoms  in  the  different  eye  muscles,  a  further  classification  according 
to  their  varying  courses  is  necessary.  We  know  of  cases  setting  in  acutely, 
frequently  comparatively  mild,  even  transient  forms;  further  of  chronic  pro- 
gressive, and  finally  of  relapsing  and  periodically  reappearing  cases. 

Whether  a  paralysis  starts  acutely,  whether  its  course  is  mild  and  regress- 
ive or  continuing,  is,  of  course,  dependent  upon  the  nature  of  the  influence, 
and  the  reparability  of  the  causal  anatomic  process.  Those  ("rheumatic") 
paralyses  which  some  writers  have  traced  to  colds,  are  famed  as  mild  cases, 
and  the  paralyses  associated  with  herpes  zoster  ophthalmicus  ought  to  per- 
mit of  a  favorable  prognosis.  The  palsies  occurring  in  tabes  and  multiple 
sclerosis,  after  lasting  a  short  time,  may  disappear  completely  and  forever. 

On  the  other  hand,  however,  these  very  ophthalmoplegias  of  tabes, 
general  paralysis  and  multiple  sclerosis  may  take  a  chronic  progressive 
course.  Several  others,  certainly  the  nuclear  forms  of  the  disease  taking  a 
like  course,  need  not  be  specially  discussed  here. 

The  recurring  form  of  paralysis  of  the  eye  muscles  occurs,  too,  in  tabes 
and  paresis.  A  second  group  of  recurring  paralyses,  which  actually  take  a 
periodic  course,  and  which  are  almost  invariably  restricted  to  an  oculomoto- 
rius,  may,  according  to  anatomic  findings,  have  their  origin  in  pressure  of 
tumors  on  the  nerve  trunk,  or  in  chronic  meningitic  and  arterio-sclerotic 

1  Strictly  speaking,  ophthalmoplegia  externa  totalis  should  be  assumed  only  when  all  external 
eye  muscles  including,  of  course,  those  innervated  by  the  abducens  and  trochlearis,  are  affected. 


n6  DISEASES  OF  THE  PERIPHERAL  NERVES 

processes  taking  place  in  the  immediate  vicinity  of  the  nerve  and  injuring  it. 
The  duration  of  the  exacerbations,  and  the  periods  of  remission  and  inter- 
mission will  naturally  be  very  different  in  the  various  cases.  At  the  time  of 
aggravation,  a  headache  on  the  side  affected  manifests  itself,  sometimes 
accompanied  by  vomiting,  in  consequence  of  which,  it  has  become  common 
to  speak  of  it  as  "migraine  ophthalmoplegique "  from  a  certain  superficial 
resemblance  in  clinical  pictures.  These  conditions,  nevertheless,  have  noth- 
ing in  common  with  genuine  migraine.  However,  it  is  said  that  even  in 
genuine  migraine  some  transient  attacks  of  eye-muscle  palsy  occur. 

Diagnosis. — The  first  problem,  though  by  no  means  always  the  easiest 
one,  is  to  find  out  definitely  whether  a  paralysis  of  the  eye  muscles  exists  at 
all,  and  what  muscles  it  affects.  For  this  the  reader  is  referred  to  the  dis- 
cussion in  the  general  section  dealing  with  diagnosis. 

In  the  second  place  we  must  try  to  discover  whether  it  is  a  supranuclear 
or  a  peripheral  paralysis  proper,  using  that  term  in  its  widest  sense.  Finally, 
and  of  most  significance  to  us  here,  we  are  confronted  by  the  most  difficult, 
only  too  often  insoluble,  question:  which  portion  of  the  peripheral  neuron  is 
the  primarily  diseased  part — is  it  the  nucleus,  the  intra-cerebral  root-fibres, 
the  intra-cranial  or  the  orbital  part  of  the  peripheral  nerve  ? 

At  first  we  must  say  a  few  words  concerning  the  differentiation  of  dis- 
ease of  the  peripheral  neurons  from  the  supranuclear  or  looking-paralysis 
(Blicklahmiing),  a  problem  the  solution  of  which  needs  special  tests. 

Generally  speaking,  in  diseases  of  the  peripheral  neuron  only,  it  is  a 
matter  of  a  true  paralysis  of  the  muscles,  while  in  supranuclear  lesions  only 
certain  functions  of  the  muscles  disappear.  Only  in  peripheral  paralysis 
is  the  muscle  equally  cut  off  from  all  inflowing  nervous  impulses.  A  supra- 
nuclear focus,  on  the  other  hand,  interrupts  only  those  pathways  which 
convey  to  a  certain  group  of  synergists  the  impulses  for  a  certain  common 
function,  so  that  the  latter  is  suspended,  while  the  same  muscles  may  remain 
normal  for  other  functions,  and  open  to  inflowing  innervation  from  other 
sources.  Thus  the  "looking  motion"  (Blickbewegung)  towards  one  side 
may  be  palsied,  while  the  co-affected  internus  is  active  in  convergent  motion. 
A  supranuclear  lesion,  however,  may  also  be  restricted  to  the  fibres  which 
convey  the  impulse  to  one  of  the  synergists.  In  that  case,  only  this  one 
muscle  is  affected,  but  only  for  the  function  concerned,  without  therefore 
being  incapacitated  for  other  functions.  In  such  cases,  just  as  in  peripheral 
paralysis,  disturbances  in  equilibrium  and  double  images  manifest  them- 
selves with  each  innervation  of  the  synergism,  which  in  one  of  its  parts  is 
injured.  Decisive  for  the  differential  diagnosis  as  to  the  supranuclear  seat 
is,  therefore,  the  proof  that  the  paralytic  symptoms  appear  only  in  certain 
functions,  while  the  same  muscles  for  other  functions  remain  normal.  One 
must  consequently  make  different  tests.  First,  the  patient  is  commanded 
to  look  in  this  or  that  direction,  or  to  fix  with  his  eye  an  object  located 


FOCAL  DIAGNOSIS  OF  OCULAR  PARALYSIS  117 

within  the  peripheral  part  of  his  field  of  vision.  Besides  these  "com- 
manded" movements,  one  should  test  the  so-called  reflex  eye  movements. 
In  the  first  place,  the  patient  is  made  to  follow  with  his  eyes  a  moving 
object — which  he  has  previously  fixed — in  all  directions;  and  secondly,  he 
is  made  to  hold  with  his  eyes  a  fixed  object  located  in  the  primary  direction 
of  his  vision,  while  his  head  is  passively  turned  in  all  directions.  In  the 
particular  case  of  a  paralysis  of  an  internus  for  the  lateral  looking  move- 
ments, his  convergence  function  should  also  be  examined,  and  vice  versa. 
In  the  majority  of  cases,  these  methods  will  suffice  to  determine  the  above 
mentioned  characteristics  of  a  supranuclear  paralysis.  In  other  cases 
different  criteria  are  required.  It  is  conceivable  that  because  of  complicated 
lesions  the  reflex  as  well  as  the  "commanded"  movements  may  be  syn- 
chronously suspended,  while  the  peripheral  neuron  is  not  diseased.  In 
that  case,  the  strictly  symmetrical  character  of  a  paralysis  may  serve  as 
evidence  of  its  supranuclear  origin.  An  absolutely  symmetrical  paralysis 
of  two  or  more  synergists,  in  which  no  disturbance  in  the  muscular  equi- 
librium could  be  detected,  does  not  occur,  aside  from  cases  of  total  paralysis, 
in  peripheral  diseases.  It  is  evident,  however,  from  the  above,  that  the 
converse  need  not  be  necessarily  true,  i.  e.,  every  paralysis  that  affects  only 
one  synergist,  or  several  unevenly,  is  not  by  reason  of  this  a  peripheral 
palsy. 

If  these  criterions  be  heeded,  the  neuron  diagnosis  will  in  most  cases  be 
successful.  To  decide  more  exactly  in  what  part  of  the  peripheral  neuron 
the  seat  of  the  lesion  is  to  be  looked  for,  and  particularly  to  decide  whether 
it  is  a  question  of  a  disease  of  the  peripheral  nerve,  the  following  points 
deserve  consideration.  In  an  isolated  disease  of  the  abducens  or  trochlear, 
this  decision  can  naturally  be  made  only  in  those  cases,  where  accompanying 
symptoms  render  possible  the  diagnosis  of  the  fundamental  anatomic 
process  and  its  seat.  Even  for  oculomotor  paralysis  this  is  frequently  the 
surest  and  often  the  only  practicable  way. 

It  was  assumed  formerly,  that,  excluding  cases  due  to  orbital  disease, 
the  paralyses  of  single  muscles  supplied  by  the  third  nerve,  were  all  caused 
by  nuclear  disease.  At  present  we  know  that  disease  of  the  nerve  trunk 
may  lead  only  to  paralysis  of  one  or  a  few  muscles,  and,  indeed,  that  this 
occurs  not  infrequently.  Isolated  ptosis  can,  in  numerous  cases,  be  traced 
to  disease  of  the  nerve  trunk.  Partial  disease  of  the  trunk,  for  example 
scattered  foci  of  disease,  have  been  proved  anatomically  in  a  number  of 
such  cases.  The  picture  of  external  oculomotor  paralysis  may  surely  be 
brought  about  in  this  way.  There  is  no  accurate  explanation  for  it.  It  is 
doubtful  whether  a  unilateral  external  oculomotor  paralysis  may  result 
from  nuclear  disease.  The  fibres  proceeding  from  the  nuclei  of  both 
oculomotor  nerves  are  subjected  to  a  partial  crossing,  so  that  each  nerve  is 
composed  of  crossed  and  uncrossed  fibres.     Just  how  both  are  distributed 


n8  DISEASES  OF  THE  PERIPHERAL  NERVES 

to  the  various  muscles,  is  a  question  which  has  not  yet  been  finally  settled. 
The  clinical  symptoms .  which  follow  the  destruction  of  one  oculomotor 
nucleus  are  not  as  yet  known  in  detail.  This  is  responsible  in  part  for  the 
great  difficulties  confronting  us  in  the  task  with  which  we  are  here  concerned. 
Only  this  may  be  said  with  certainty,  that  a  total  paralysis  of  all  branches 
of  the  oculomotorius  will  have  to  be  localized  distally  from  the  partial 
crossing  of  the  fibres,  in  the  majority  of  cases,  basally. 

Our  knowledge  concerning  the  localization  of  ophthalmoplegia  interna 
is  also  very  incomplete.  It  is  pointed  out  that  a  connection  of  an  ophthal- 
moplegia interna  with  a  paralysis  of  the  inferior  oblique  muscle  indicates 
orbital  disease,  since  the  short  root  of  the  ciliary  ganglion  which  contains 
the  oculomotor  fibres  of  the  inner  eye  muscle  originates  from  the  nerve 
branch  destined  for  the  inferior  oblique. 

Unfortunately  we  know  of  no  other  pathognomonic  classification  of  the 
symptoms,  which  would  be  even  partially  accurate  for  certain  places  in  the 
peripheral  pathway.  When  several  different  eye  muscles  are  affected 
simultaneously,  it  denotes  with  certainty  a  basal  or  orbital  seat.  The  cases 
taking  a  chronic  progressive  course  most  frequently  are  the  result  of  nuclear 
disease;  those  taking  an  acute  and  mild  course,  and  the  relapsing  and 
periodic  cases,  are  generally  due  to  peripheral  nerve  disturbance. 

The  differentiation  of  certain  myopathic  ophthalmoplegias  from  neuro- 
pathic forms  is  frequently  possible  only  by  taking  into  consideration  the  dis- 
ease picture  as  a  whole.  (Cf.  especially  the  chapter  on  myasthenia.) 
Ophthalmoplegia  interna,  occasioned  by  the  effect  of  a  blow  on  the  eye- 
ball, is  probably  myopathic. 

The  prognosis  of  an  ocular  paralysis  should  always  be  made  with  great 
reserve.  Above  everything  else  the  fundamental  disease  must  be  considered. 
Even  in  those  cases  in  which  it  is  possible  to  diagnose  the  pathologic-anatomic 
process  and  to  recognize  it  as  remediable,  it  will  not  always  be  certain  whether 
the  changes  this  has  occasioned  in  the  nerve  are  likewise  adjustable.  Con- 
cerning their  nature  and  gravity  a  decision  is  frequently  impossible.  But 
only  the  inveterate  cases  and  those  taking  a  chronic  progressive  course  are 
hopeless  from  the  beginning.  A  favorable  prognosis  is  possible  in  the  acute 
cases,  occurring  in  connection  with  infectious  diseases  and  with  a  number  of 
toxic  diseases  (botulism).  Some  other  important  points  for  the  prognosis 
have  been  mentioned  in  preceding  paragraphs. 

Therapy. — There  can  really  be  no  question  of  special  therapeutics  for 
paralyses  of  the  eye  muscles.  Whenever  possible  the  treatment  should  be 
aimed  at  the  fundamental  disease.  In  all  other  respects  one  should  proceed 
according  to  principles  laid  down  for  the  treatment  of  paralyses  in  general. 
The  use  of  the  stabile  cathode  on  the  closed  eyelids  of  the  diseased  eye  should 
be  tried.  The  inactive  electrode  is  placed  on  the  neck.  From  an  authori- 
tative source  comes  the  suggestion  that  a  few  sweat  baths  be  prescribed  in 


THE  TRIGEMINAL  NERVE  id 

those  acute  cases,  which  presumably  are  due  to  an  infectious  cause  or  ex- 
posure to  cold. 

Later,  exercises  to  improve  vision,  and  in  the  residual  stage  oculistic  and 
especially  operative  measures  for  the  adjustment  of  the  disturbances  of 
vision,  will  have  to  be  considered. 

4.  The  Trigeminal  Nerve 

The  trigeminal  nerve  arises,  like  a  spinal  nerve,  from  the  central  organ 
with  an  anterior  motor  and  a  posterior  sensory  root.  The  posterior  root 
enters  the  Gasserian  ganglion.  Distally  from  it  the  motor  root  unites  with 
the  third  of  the  sensory  nerve  branches  proceeding  from  the  ganglion ;  the 
first  two  of  these  nerve  branches  consequently  remain  purely  sensory. 
Besides  combined  disease  of  both  roots,  there  may  be  disease  of  the  sensory 
part  alone.  Cases  of  purely  motor  trigeminal  paralysis  (masticatory  facial 
paralysis)  are  also  on  record.  Besides  the  affections  of  the  chief  trunk, 
paralyses  of  single  branches  have  been  observed. 

Trigeminal  paralysis  usually  makes  its  appearance  as  a  partial  symptom 
of  other  disease  pictures;  it  very  rarely  occurs  isolated. 

Its  most  frequent  cause  is  basal  disease.  In  basal  syphilis,  in  tumors  of 
the  base  of  the  brain  or  skull,  and  after  fracture  of  the  skull,  one  may  occa- 
sionally observe  isolated  trigeminal  paralyses,  though  even  in  these  cases  it  is 
seen  more  frequently  in  conjunction  with  palsies  of  other  cranial  nerves. 

Of  diseases  of  single  branches,  that  of  the  ramus  ophthalmicus,  due  to 
morbid  changes  in  the  superior  orbital  fissure  or  inside  the  orbit,  is  observed 
most  commonly. 

In  a  later  chapter  it  will  be  shown  that  neuralgia  may  be  followed  by 
symptoms  of  lost  function  in  single  trigeminal  branches. 

During  the  last  few  years,  extirpation  of  the  Gasserian  ganglion  in  grave 
cases  of  neuralgia  has  furnished  repeatedly  an  opportunity  for  studying  the 
symptoms  of  trigeminal  paralysis. 

A  lesion  of  the  motor  part  gives  rise  to  paralysis  of  the  muscles  of  masti- 
cation. It  may  be  both  seen  and  felt  how  the  temporal  and  masseter  mus- 
cles on  the  affected  side  fail  to  contract,  either  wholly  or  partially,  when 
chewing  and  biting,  and  how  they  gradually  atrophy.  The  lower  jaw  reflex 
is  also  abolished  on  that  side.  The  lower  jaw,  as  a  result  of  the  paralysis  of 
the  pterygoids,  can  not  be  moved  towards  the  healthy  side,  it  tends  rather, 
during  chewing,  to  deviate  towards  the  affected  side.  In  bilateral  paralysis 
the  jaw  hangs  down  loose;  it  can  be  lifted  only  with  the  hand  and  an  habitual 
dislocation  may  develop. 

Paralyses  of  the  other  muscles  supplied  by  the  motor  trigeminus  (mylo- 
hyoid, anterior  belly  of  the  digastric,  tensor  tympani  and  tensor  veli  palati) 
do  not  occasion  very  significant  symptoms. 

The  disturbances  of  sensation  affect,  according  to  the  seat  of  the  lesion, 
the  whole  innervation  area  or  that  of  single  branches.     For  a  better  under- 


120  DISEASES  OF  THE  PERIPHERAL  NERVES 

standing  of  the  clinical  pictures  and  particularly  of  the  rather  far  reaching 
adjustability  or  possibility  of  compensation,  it  is  exceedingly  important  to 
know,  that  the  innervation  areas  of  the  right  and  left  nerves,  the  areas  of 
the  single  branches  among  themselves,  and  finally  the  areas  of  the  trigeminus 
and  that  of  the  neighboring  cervical  nerves  may  overlap  one  another 
extensively. 

It  should  further  be  remembered,  that  the  innervation  area  of  the 
trigeminus  is  not  confined  to  the  skin  of  the  face.  Of  those  parts  that  can  be 
examined,  the  first  branch  supplies  the  cornea  and  conjunctiva  as  well  as  the 
anterior  and  upper  part  of  the  nasal  cavity;  the  second,  the  remaining  part 
of  the  nasal  cavity,  the  upper  jaw  and  the  mucous  membrane  of  the  palate 
and  upper  lip ;  the  third,  the  lower  jaw  and  the  mucous  membrane  of  the 
cheek,  the  tongue  and  the  lower  lip. 

Besides  the  sensory  symptoms  of  loss  of  function,  pains  frequently  occur, 
which,  especially  in  compression  of  the  nerve  trunk,  may  be  neuralgic  and 
be  projected  into  the  peripheral  extension  area. 

Together  with  the  conduction  of  sensibility,  the  conjunctival  and  corneal 
as  well  as  the  sneezing  and  palatal  reflexes,  which  are  mediated  by  the  sensory 
trigeminus  path,  are  interfered  with.  The  functions  of  the  lachrymal  and 
salivary  glands,  especially  those  of  the  former,  suffer  from  the  loss  of  a  great 
part  of  the  reflex  irritations,  that  stimulate  their  secretion.  As  a  result  of 
this  and  of  other  disturbances  in  the  region  of  the  smaller  mucous  glands, 
there  occurs  a  certain  dryness  of  the  conjunctiva  and  the  mucous  membrane  of 
the  nose,  which  may  even  in  some  cases,  it  is  said,  impair  the  sense  of  smell. 
The  real  secretory  nerves  of  the  glands  mentioned  are  not,  however,  generally 
affected.  A  glance  at  the  diagram  (Fig.  28)  will  show  that  these  fibres  do 
indeed  mingle  with  branches  of  the  trigeminus  that  are  directed  towards  the 
periphery  and  that  in  the  last  part  of  their  course  they  take  the  same  course  as 
these  branches.  Lesion  of  them  in  the  trigeminus  area  would  therefore  be 
possible  only  if  those  terminal  branches  should  be  subjected  to  an  injury. 
As  a  rule,  then,  a  true  paralysis  of  glandular  secretion  does  not  occur,  and  it 
may  be  expected  that  the  psychic  emotional  secretion  especially  will  go  on 
quite  undisturbed. 

Fig.  28  gives  information  also  concerning  the  relation  of  the  peripheral 
pathway  of  taste  to  the  trigeminus. 

It  must  even  be  emphasized  here,  and  we  will  be  confronted  by  it  again 
when  studying  facial  paralysis,  that  without  doubt  individual  variations  in  the 
course  of  the  taste  fibres  occur  and,  indeed,  it  seems  that  their  relations  to  the 
trigeminus  are  less  regular  than  those  comparatively  constant  ones  to  the 
facial  nerve.  The  fibres  emanating  from  the  anterior  two-thirds  of  the 
tongue  enter  almost  regularly  the  lingual  nerve,  which  they  soon  leave, 
however,  in  order  to  enter  the  facial  trunk  with  the  chorda.  They  leave 
it  in  the  vicinity  of  the  geniculate  ganglion,  mostly  via  the  large  superficial 


THE  TRIGEMINAL  NERVE  121 

petrosal,  enter  thereafter  into  the  second  trigeminal  branch  and  with  the 
latter  reach  the  chief  trunk.  But  after  careful  observation  it  must  be 
admitted  that  there  is  a  possibility  of  the  small  petrosal  taking  over,  in  a  few 
cases,  the  gustatory  fibres  of  the  chorda  from  the  facial  trunk  and  taking 
them  to  the  trigeminal  trunk  by  means  of  the  third  branch.  This  covers 
the  most  important  possibilities  of  a  taste  disturbance  in  trigeminal  disease. 
On  the  other  hand  it  would  seem  certain  that  a  lesion  of  the  trigeminal  trunk 
need  not  necessarily  lead  to  disturbances  of  taste.  In  such  cases  it  is 
assumed  that  the  fibres  of  the  chorda  after  their  usual  course  reach   the 


I.  trigeminus 


lingualis  u  pubmaxittarip 


Fig.  28. — Scheme  of  the  relations  between  the  trigeminal,  the  facial  and  the  glosso-pharyngeal. 

Taste  fibres;     Secretory  fibres  for  sublingual  and  submaxillary  glands;      +  +  +  + 

Secretory  fibres  for  tear  glands.     {Simplified  from  Koster.) 


glosso-pharyngeal  trunk  by  anastomosis  through  the  facial  and  with  it  enter  the 
brain.  That  the  chorda  should  not  contain  any  taste  fibres  at  all,  may 
occur  as  a  great  rarity.  In  that  case  they  either  reach  the  trigeminal  trunk 
directly  through  the  lingual  nerve  or  the  lingual  contains  no  taste  fibres  and 
the  glosso-pharyngeal  innervates  the  entire  half  of  the  tongue  directly. 
So  far  we  have  spoken  only  of  the  gustatory  innervation  of  the  anterior 
half  of  the  tongue,  usually  effected  through  the  chorda.  The  taste  nerves  of 
the  posterior  part  of  the  tongue,  which  usually  belong  to  the  lingual  branches 
of  the  glosso-pharyngeal,  do  not,  perhaps,  always  take  their  further  course  to 
the  brain  through  the  glosso-pharyngeal  trunk,  but  sometimes,  by  means  of 
the  Jacobson  anastomosis  (the  tympanic,  resp.  the  small  superficial  petrosal) 


122  DISEASES  OF  THE  PERIPHERAL  NERVES 

also  enter  the  trigeminal  trunk.  This  is  corroborated  by  the  experience  of 
aurists  in  cases  of  lesion  of  the  tympanic  nerve,  in  connection  with  the 
hemiageusia  occurring  occasionally  in  diseases  of  the  trigeminal  trunk. 

Finally,  it  would  seem  that  occasionally  the  chorda  supplies  the  whole 
half  of  the  tongue  with  taste  fibres. 

In  view  of  the  great  variability  of  the  clinical  pictures,  the  discussion  of  all 
these  probabilities  could  not  be  omitted.  The  careful  study  of  each  new 
individual  case  is  urged  for  the  sake  of  acquiring  a  better  and  clearer  insight 
into  these  complicated  conditions. 

Of  greatest  interest  among  the  trophic  disturbances  are  herpes  zoster, 
to  be  discussed  in  the  chapter  on  neuralgia,  and  the  so-called  neuroparalytic 
keratitis.  The  more  or  less  serious  inflammations  in  corneal  anaesthesia, 
which  are  borne,  however,  with  remarkable  ease,  must  certainly  be  closely 
related  to  external  injuries,  from  which  the  cornea,  on  account  of  its  insen- 
sibility, is  not  sufficiently  protected.  Nevertheless  the  foremost  authorities 
agree  that  the  pathogenesis  of  this  keratitis  is  still  a  very  complicated  prob- 
lem.    Furthermore  not  every  corneal  anaesthesia  leads  to  keratitis. 

Diagnosis. — Only  the  differential  diagnosis  needs  to  be  discussed 
particularly. 

The  motor  disturbances,  when  occurring  isolated  and  without  accom- 
panying defects  of  sensibility,  may  make  the  diagnosis  difficult. 

The  myopathic  conditions  of  weakness  and  paralysis  of  the  muscles 
of  mastication — such  as  are  found  in  myasthenia,  in  very  rare  cases  in  the 
common  forms  of  muscle  dystrophy,  but  occur  more  regularly  in  the  advanced 
dystrophies  of  those  affected  with  Thomsen's  disease — manifest  themselves 
bilaterally.  The  characteristic  general  aspects  of  the  affections  in  question 
will  always  facilitate  the  diagnosis. 

The  central,  supranuclear  palsies  of  the  masticatory  muscles  affect  both 
sides.  They  are  accompanied  by  exaggerated  reflexes.  R.  D.  is,  of  course, 
always  absent.  The  disturbance  of  mastication  is  a  partial  symptom  of 
the  typical  general  picture  of  pseudo-bulbar  paralysis.  Therefore  the 
diagnosis  is  comparatively  easy  in  these  cases  also. 

Difficulties  in  differentiation  may  arise  in  cases  of  nuclear  and  intra- 
cerebral radicular  paralyses,  which  bear  the  character  of  peripheral  paralysis, 
without  having  any  features  of  their  own,  to  distinguish  them  from  diseases 
of  the  peripheral  nerves.  Such  cases  occur  in  focal  diseases  of  the  brain- 
stem, in  polioencephalitis,  in  bulbar  paralysis  and  in  tabes.  In  practice, 
consideration  of  all  the  symptoms  together  will  show  in  most  of  even  these 
cases,  that  a  peripheral  nerve  disease  is  out  of  the  question. 

The  disturbances  of  sensation,  which  in  their  distribution  correspond 
exactly  to  the  innervation  area  of  the  trigeminus  or  one  of  its  main  branches, 
indicate  at  once  a  disease  of  the  peripheral  nerve.  The  fibres  entering 
the  pons  are  distributed  over  the  long  cell  column  of  the  sensory  nucleus 


TRIGEMINAL  PARALYSIS.     THE  FACIAL  NERVE  123 

of  the  trigeminal  which  extends  down  to  the  cervical  spinal  cor4-  If  a 
focal  lesion  within  the  central  organ  affects  part  of  this  extended  root  area, 
there  result  disturbances  in  sensibility  of  a  peculiar,  segmental  character; 
namely,  stripe-like  anaesthesias,  which  are  arranged  concentrically  like  the 
layers  of  an  onion  about  the  middle  of  the  face.  Such  a  zone  always  belongs 
to  the  areas  of  several  peripheral  nerve  branches.  An  anaesthesia  of  an 
entire  trigeminal  area  through  intra-cerebral  lesion  can  be  thought  of  only 
in  those  cases  in  which  a  focus  has  affected  the  fibres  already  united  in  a 
bundle,  immediately  before  their  exit  from  the  pons.  It  is  furthermore  of 
value  to  know,  that  in  a  few  cases  of  pontile  focal  trouble,  anaesthesias  have 
been  observed  which  corresponded  almost  exactly  to  those  of  an  area  of 
an  ophthalmic  nerve. 

The  anaesthesias  in  diseases  of  the  cerebrum  and  in  hysteria  are  never 
confined  very  strictly  to  a  trigeminal  area. 

There  can  be  no  question  of  a  special  prognosis  or  therapeutics  in 
trigeminal  paralysis. 

5.  The  Facial  Nerve 

The  facial  nerve  perhaps  more  often  than  any  other  peripheral  nerve  is 
affected  alone. 

Of  eminent  importance  in  the  aetiology  of  facial  palsy  is  exposure  to 
cold.  Many  authors  attribute  almost  three-quarters  of  all  cases  to  it.  To 
be  sure,  patients  remarkably  often  declare  that,  for  instance,  they  looked 
out  of  the  window  in  a  heated  condition,  thus  exposing  the  face  to  cold 
draughts,  and  that  on  the  next  day  the  facial  palsy  appeared,  etc.  But 
it  is  questionable,  to  say  the  least,  in  how  many  of  these  cases  a  cold 
is  the  direct  cause  of  the  disease.  Recent  investigations  seem  to  favor  the 
assumption  that  in  not  a  few  cases  an  acute,  if  slight,  catarrh  of  the  middle 
ear  may  be  the  connecting  link  between  cause  and  effect. 

Be  that  as  it  may,  in  any  case  the  diseases  of  the  middle  and  inner  ear 
and  of  the  petrous  portion  of  the  temporal  bone  are  among  the  most  frequent 
and  most  important  causes  of  facial  palsy.  The  thin  bone,  which  divides 
the  nerve  canal  from  the  middle  ear,  in  many  cases  is  defective  in  one  or 
more  places,  so  that  the  nerve  is  in  contact  with  the  mucous  membrane  of 
the  tympanum.  It  is  obvious  from  this,  that  even  very  slight  disease  of  the 
tympanum,  which  both  the  patient  and  the  physician  are  very  apt  to  over- 
look, may  lead  to  paralysis — a  fact  which  in  every  case  should  prompt  a 
careful  expert  examination  of  the  ear.  It  must  also  be  remembered  that 
the  palsy  may  continue  even  after  the  inflammation  of  the  ear  has  entirely 
ceased.  Serious  and  particularly  purulent  inflammations,  and  carious 
processes  on  the  petrous  portion  of  the  temporal  bone  may  injure  the  nerve 
in  various  ways. 


i24  DISEASES  OF  THE  PERIPHERAL  NERVES 

In  the  third  place,  traumatic  causes  must  be  mentioned.  At  times, 
the  facial  is  injured  in  an  operation  upon  the  ear,  or  the  nerve  has  to  be 
sacrificed  in  some  indispensable  radical  operation.  Other  typical  cases  of 
traumatic  facial  palsy  are  those  due  to  fracture  of  the  skull,  and  the  obstetric 
palsies  of  newly  born  infants,  in  which  cases,  it  is  usually  an  injury  of  extra- 
cranial branches  occasioned  either  by  pressure  of  the  mother's  pelvis,  or  by 
the  forceps  of  the  physician.  The  peripheral  terminal  arborizations  of  the 
nerve  over  the  face  suffer  lesions  very  frequently  from  cuts  and  blows. 

The  last  setiological  group  of  importance  contains  those  palsies  occasioned 
by  basal  diseases,  among  which  meningitis  of  all  kinds  and  new  growths 
in  this  region  play  the  chief  role. 

Of  less  frequent  occurrence  are  lesions  of  the  nerves  in  cases  of  adjoining 
inflammatory  diseases  located  outside  the  cranium,  such  as  parotitis. 

In  many  cases  facial  palsy  has  been  ascribed  to  some  preceding  infectious 
disease,  such  as  diphtheria  and  influenza,  to  toxic  processes,  and  to  metabolic 
diseases,  especially  diabetes.  A  recent  syphilis  is  said  to  predispose  to 
facial  palsy.  (Tertiary  syphilis  causes  basal  palsies  by  meningitis.)  Fre- 
quently, however,  investigation  does  not  reveal  such  predisposing  factors. 
Herpes  zoster  of  the  trigeminal  area  may  be  accompanied  by  a  facial  palsy. 

It  is  questionable,  whether  hereditary  neuropathic  affections  have  any 
significance.  Some  rare  observations  seem  to  support  the  theory  of  predis- 
position in  an  entire  family  to  facial  palsy.  Of  the  relapsing  forms  we  shall 
speak  later. 

Facial  palsy  appears  as  a  congenital  malformation,  usually  affecting  both 
sides,  frequently  associated  with  palsy  of  the  eye  muscles  and  with  other 
malformations.  In  the  majority  of  cases  it  is  doubtless  due  to  intra-cerebral 
disease.  The  accompanying  disturbances  in  the  oculomotor  apparatus  may 
bear  the  character  of  a  supranuclear  or  a  "looking"  paralysis.  Like 
myopathic  palsy,  nuclear  facial  palsy,  in  which  very  likely  must  be  included 
that  occurring  in  cranial  tetanus  and  tabes,  is  here  of  interest  only  for  the 
purposes  of  differential  diagnosis. 

Clinical  Picture 

In  a  recent  facial  palsy,  all  mobility  in  the  affected  half  of  the  face  is  more 
or  less  abolished.  As  a  result  of  the  flaccidity  and  paralysis  of  the  muscula- 
ture, the  wrinkles  on  the  affected  side  are  smoothed  out,  the  forehead  is 
smooth,  and  the  nasolabial  crease  not  very  marked.  The  corner  of  the  mouth 
and  the  lower  eyelid  hang  down  a  little.  The  mouth,  as  a  whole,  is  slightly 
drawn  toward  the  opposite  side.  The  palpebral  fissure  is  wider  than  on  the 
sound  side.  In  every  expressive  movement,  wrinkling  the  brows,  showing 
the  teeth,  pursing  the  lips,  laughing,  etc.,  only  the  sound  side  is  innervated. 
When  attempting  to  close  the  eyelids,  the  upper  lid  is  lowered  a  trifle  on 


PERIPHERAL  FACIAL  PALSY 


125 


account  of  the  flabbiness  of  the  levator  palpebral,  but  it  is  impossible  com  _ 
pletely  to  close  the  eyelids,  and  the  palpebral  fissure  remains  half  open 
(lagophthalmos).  In  this  attempt,  the  upward  rotation  of  the  ball,  physio- 
logically associated  with  the  impulse  to  close  the  lid,  becomes  plainly 
visible.  In  healthy  individuals  this  can  be  observed  only  by  mechanically 
preventing  the  closing  of  the  eyelids,  when  the  subject  is  strongly  trying  to 
close  them  (Bell's  phenomenon).  It  may  be  remarked  in  passing,  that 
sometimes  the  closing  of  the  lids  will  be  more  complete  during  sleep  than  the 
patient  could  ever  produce  while  awake;  and,  on  the  other  hand,  that  some- 
times the  fissure  is  larger  during  sleep  than  during  a  strong  volitional  attempt 


Fig.  29. — Paralysis  of  the  facial  at  the  right 
side  during  rest.     (Leipsic  Medical  Clinic.) 


Fig.  30. — Paralysis  of  the  facial  at  the  right 
side  when  trying  to  whistle.  (Leipsic  Medical 
Clinic.) 


to  close  them.  One  may  usually  convince  one's  self  of  a  unilateral  palsy  of  the 
platysma  by  asking  the  patient  to  draw  his  under  lip  as  far  as  possible  down- 
ward. The  paralysis  of  the  posterior  auricular  branch  coursing  to  the  ear 
muscles  and  to  the  occipital  muscle,  and  of  the  branches  destined  for  the 
stylohyoid  and  the  posterior  belly  of  the  digastric,  manifest,  as  a  rule,  no 
symptoms  worthy  of  mention. 

The  direct  consequences  of  the  disturbance  of  the  mobility  in  unilateral 
facial  palsy  are  of  little  importance  from  a  practical  standpoint.  The  patient 
can  not  whistle;  when  he  attempts  to  blow  out  a  candle,  the  air  escapes,  with 
but  feeble  pressure,  through  the  corner  of  the  mouth  on  the  affected  side. 
If  there  is  an  abundant  secretion  of  saliva,  the  imperfect  closing  of  the  mouth 


126  DISEASES  OF  THE  PERIPHERAL  NERVES 

may  become  noticeably  annoying,  as  the  saliva  may  flow  out  of  the  mouth 
on  the  affected  side. 

Of  much  greater  importance  is  a  lasting  lagophthalmos,  which  regularly 
causes  the  eye  to  weep  considerably,  quite  often  results  in  an  obstinate  con- 
junctivitis and  once  in  a  while  even  leads  to  graver  inflammations  of  the  eye. 

In  the  not  very  rare  bilateral  paralysis  (diplegia  facialis),  the  motor 
loss  is  immediately  and  very  painfully  perceived.  Speech  is  very  much 
impaired  by  the  loss  of  the  labial  sounds.  The  patient  cannot  expectorate 
and  can  blow  only  feebly.  The  flabbiness  of  the  cheeks,  which  in  chewing  do 
not  touch  the  teeth,  makes  eating  difficult.  The  saliva  cannot  be  properly 
retained  in  the  mouth,  but  flows  out  over  the  lower  lip.  The  entire  face 
is  rigid  like  a  mask,  and  because  of  the  drooping  of  the  lower  eyelids  and  lips, 
exceedingly  distorted  (Fig.  31). 

Coincident  with  the  symptoms  of  facial  palsy,  disturbances  in  secretion  of 
perspiration  frequently  manifest  themselves,  in  which  case,  both  hyperhi- 
drosis  and  anhidrosis  may  occur.  This  alone  makes  it  evident  that  a  strict 
parallel  does  not  exist  between  the  injury  of  motor  fibres  and  those  taking  the 
same  course  which  subserve  the  production  of  perspiration.  No  diagnostic 
interest  attaches  to  these  manifestations.  The  hyperhidrosis  will  usually 
be  noticed  when  chewing,  especially  when  chewing  foods  containing  acids. 

We  now  have  to  consider  some  accompanying  symptoms,  which  mani- 
fest themselves  only  if  the  facial  is  injured  in  certain  places  in  its  course. 
(Cf.  diagram  on  p.  121,  Fig.  28.) 

From  the  point  where  the  facial  nerve  leaves  the  brain,  secretory  fibres 
subserving  the  lachrymal  glands  take  their  course  through  the  nerve  trunk 
to  the  geniculate  ganglion,  where  they  leave  it  through  the  large  superficial 
petrosal  nerve.  A  lesion  of  the  facial  nerve  within  the  limits  just  outlined 
regularly  leads  to  disturbance  in  the  secretion  of  tears,  causing  either  a 
decrease  or  cessation,  or  even  a  morbid  increase  of  the  secretion  (Koster). 

As  this  symptom  is  diagnostically  important  a  short  account  of  the  technic 
for  examination  follows.  Strips  of  about  20  cm.  in  length  and  1  cm.  in 
width  are  cut  from  blotting  paper  and  one  end  of  each  turned  down  a  trifle 
making  a  hook.  By  means  of  these  hooks  one  strip  is  hung  into  the  con- 
junctival sac  of  each  of  the  two  lower  eyelids,  which  should  first  be  somewhat 
dried  in  order  to  remove  the  too  ample  secretions  of  the  mucous  membrane, 
and  their  gradual  permeation  is  then  awaited.  The  lachrymal  glands  may 
be  stimulated  to  action  by  properly  inserting  a  small  brush  well  up  into  each 
nostril.  This  test  is  continued,  until  the  strips,  which  when  fully  per- 
meated should  be  exchanged  for  fresh  ones,  are  no  longer  permeated,  in  other 
words,  until  the  lachrymal  glands  are  "pumped  out."  The  functional 
capacity  of  both  sides  is  then  judged  according  to  the  length  of  the  per- 
meated space  on  the  strips.  Minor  differences  (up  to  3  or  4  cm.  of  the  blot- 
ting paper)  occur  even  in  normal  conditions.     Now,  in  facial  palsy  we  find 


■PERIPHERAL  FACIAL  PALSY 


127 


in  the  corresponding  localized  cases  on  the  affected  side  a  cessation  or  a 
large  decrease,  or  an  excessive  increase,  in  the  secretion  of  tears.  If  a  marked 
difference  between  both  sides  is  at  once  obvious,  say  if  the  secretion  should 
be  wholly  lacking  on  one  side,  the  test  need  not  be  so  protracted,  but  in 
doubtful  cases  the  "pumping  out"  which  may  take  an  hour  or  more,  must 
be  patiently  awaited.  Worthy  of  mention  is  the  fact  that  the  eye  on  the  side 
affected  with  paralysis  may  weep,  even  when  the  secretion  of  the  lachrymal 
gland  has  ceased.  The  comparatively  tenacious  secretion  of  the  palpebral 
conjunctiva,  however,  is  sufficient 
only  for  permeating  at  the  most  about 
1  cm.  of  the  strip  of  blotting  paper. 

The  older  investigators  assumed 
that  motor  fibres,  too,  left  the  facial 
nerve  by  way  of  the  large  superficial 
petrosus,  to  enter  the  palate.  Today 
we  know  that  the  facial  does  not  in- 
nervate the  palate.  Consequently  a 
paralysis  of  the  palate  is  not  a  part  of 
the  clinical  picture  of  facial  paralysis. 
The  former  erroneous  notion  can 
partially  be  accounted  for  by  the  fact 
that  the  oblique  position  of  the  uvula, 
which  is  frequent  even  in  healthy 
persons,  was  falsely  regarded  as  a 
sign  of  a  unilateral  palsy  of  the 
palate. 

Taste  fibres  which  have  been  in- 
troduced into  the  facial  more  distally, 
through  the  chorda  tympani,  leave  it 
through  the  large  superficial  petrosal, 
coursing  to  the  second  trigeminal  branch.  In  an  affection  of  the  facial  nerve 
in  its  course  between  the  geniculate  ganglion  and  the  junction  of  the  chorda, 
disturbances  of  taste  are  evidenced,  corresponding  to  the  innervation  area  of 
the  chorda,  in  the  anterior  two-thirds  of  the  half  of  the  tongue  on  the  same 
side. 

It  must  be  mentioned,  though  it  does  not  materially  affect  the  practical 
worth  of  the  above  statements,  that  in  very  rare  cases  taste  innervation 
takes  a  somewhat  different  course.  It  occasionally  happens  that  the 
chorda  fibres  innervate  gustatorily  the  entire  half  of  the  tongue;  but  it  has 
also  been  noted,  that  in  spite  of  the  destruction  of  the  chorda,  taste  disturb- 
ances were  wholly  lacking. 

In  the  area  in  which  the  chorda  fibres  course  in  the  nerve  trunk  a  very 
fine  small  branch,  whose  function  is  not  known  and  which  anastomoses  with 


Fig.  31. — Bilateral  congenital  paralysis  of 
the  facial.  Attempt  to  close  the  eyes.  Mal- 
formation of  the  ears.     (Leipsic  Medical  Clinic) . 


128  DISEASES  OF  THE  PERIPHERAL  NERVES 

the  small  superficial  petrosal  nerve  and  a  motor  twig,  taking  its  course  to- 
wards the  stapedius  muscle,  are  separated.  It  would  therefore  have  to  be 
assumed,  that  the  symptoms  of  paralysis  of  this  muscle  exist  in  every  facial 
paralysis  situated  above  the  point  of  separation  of  its  twig.  Indeed,  in- 
creased auditory  sensibility,  keenness  of  hearing  (hyperakusia),  has  been 
sometimes  regarded  as  a  symptom  of  stapedius  palsy.  But  this  symptom  is 
so  inconstant,  so  difficult  to  determine  correctly,  and  so  questionable  in  its 
meaning,  that  one  has  to  relinquish  it  for  all  purposes  of  determining  the 
diagnosis. 

Fibres  for  the  submaxillary  and  sublingual  glands  take  their  course 
down  the  nerve  trunk  to  where  the  chorda  tympani  leaves,.  In  conformity 
with  this,  in  many  instances,  disturbances  of  the  secretion  of  these  glands 
are  observed,  manifesting  themselves  chiefly  in  a  decrease,  though  some- 
times in  an  increase  in  the  secretion  of  saliva.  For  determining  these  things, 
we  can  only  avail  ourselves  of  the  very  inadequate  method  of  asking  the 
patient  to  raise  his  tongue,  to  dry  the  floor  of  his  mouth  and  then  to  examine 
and  compare  the  two  sublingual  carunculae. 

More  pronounced  trophic  and  vasomotor  disturbances  are  very  rare. 
Sensory  disturbances  do  not,  as  a  rule,  enter  into  the  clinical  picture  of 
facial  paralysis. 

According  to  recent  investigations  the  chorda  is  supposed  to  contain, 
besides  the  fibres  of  taste,  a  few  sensory  fibres,  whose  further  course  to 
the  central  organ  is  unknown.  It  is  true  that  occasionally  a  very  slight 
deadening  of  the  sensibility  is  detected  in  the  innervation  area  of  the  chorda  dur- 
ing facial  paralysis.  Perhaps  slight  disturbances  of  the  sensibility  in  the  area 
of  the  skin  of  the  face  would,  with  proper  attention,  be  detected  more  fre- 
quently than,  according  to  general  belief,  they  occur.  In  such  instances 
it  is  questionable  whether  it  is  always  a  true  complication  with  trigeminal 
disease,  or  whether  sensory  fibres  may  not  also  enter  into  the  facial  trunk. 
Peripheral  anastomoses  between  the  sensory  and  motor  facial  nerves  are 
known  to  exist  and  central  relations  between  the  facial  roots  and  the  bulbar 
terminals  of  the  trigeminal  fibres  have  been  reported. 

Besides  disease  of  the  chief  trunk,  paralyses  of  single  branches  also  occur. 
Cuts  and  blows  in  the  face  are  their  most  frequent  cause.  In  its  course 
through  the  middle  ear,  the  chorda  is  sometimes  affected  alone  as  a  result 
of  an  otitis  media.  It  is  occasionally  destroyed  in  the  course  of  an  opera- 
tion. As  a  result,  corresponding  disturbances  of  taste  and  of  the  secretion 
of  saliva  set  in.  Isolated  paralyses  of  the  large  superficial  petrosal  have 
been  observed  after  operative  lesions  in  which  the  Gasserian  ganglion  has 
been  extirpated;  the  scars  following  such  procedure  may  also  injure  the 
nerve.  We  have  already  noted  in  basal  syphilis  the  characteristic  symptoms 
of  its  isolated  affection — taste  disturbances  in  the  chordal  area  and  cessa- 
tion of  lachrymal  secretion. 


THE  COURSE  OF  PERIPHERAL  FACIAL  PALSY  129 

The  Course  of  the  Disease 

The  prodromal  symptoms  which  are  sometimes  observed,  may  be 
traced  back  in  part  to  the  original  disease,  such  as  fever,  headache  and  ear- 
ache; some  of  them,  such  as  taste  paresthesia,  slight  clonic  spasms,  some- 
times perhaps  even  pains  in  the  face,  point  to  a  lesion  of  the  nerve  as  already 
present. 

Usually  the  palsy  sets  in  acutely.  A  gradual  onset  may  sometimes  occur, 
most  frequently  in  basal  compression  of  the  nerve. 

The  course  of  the  disease,  in  the  majority  of  cases,  is  favorable;  the 
mobility  of  the  palsied  area  is  restored  more  or  less  completely.  In  many 
cases  the  restoration  of  the  palsied  area  is  not  at  all  uniform.  Some  muscles 
are  more  completely  and  sooner  able  to  perform  their  functions  than  others. 
The  course  of  the  other  functional  disturbances  does  not  always  correspond 
precisely  to  that  of  the  palsy.  The  disturbances  in  the  secretion  of  perspi- 
ration accompanying  facial  paralysis,  are,  as  stated  above,  inconstant  from 
the  very  first;  they  may  be  either  paralytic  or  irritative  and  are  frequently  re- 
stored rather  quickly.  Similar  conditions  prevail  as  regards  disturbances 
of  the  salivary  secretion — though,  it  must  be  added,  only  in  so  far  as  we  are 
able  to  judge.  They,  too,  are  inconstant,  even  when  the  lesion  takes  place 
in  the  area  through  which  the  fibres  that  excite  the  glands  take  their  course, 
and  they  also  may  be  restored  before  the  paralysis.  Taste  disturbances 
are  often  only  partial,  appearing  to  affect  only  a  part  of  the  taste  qualities, 
and  they  too  may  disappear  before  the  palsy.  One  gains  the  impression, 
that  possibly  the  taste  function  quite  like  that  of  sensibility  in  general  is, 
in  diseases  of  peripheral  nerves,  less  subject  to  injury. 

Recovery  in  the  lighter  cases  is  usually  complete,  in  grave  cases  usually 
defective.  The  contractures  and  associated  movements  manifesting  them- 
selves in  cases  in  which  the  restoration  has  been  only  partial  are  more  trouble- 
some than  the  residuum  of  the  palsy. 

The  facial  contracture  is  conditioned  upon  a  peculiar  shrinking  process 
of  the  paretic  muscles,  thus  distinguishing  itself  from  other  contractures  in 
peripheral  palsy,  which  are  produced  by  the  shortening  of  antagonists  not 
affected  by  the  paralysis. 

The  anatomic-physiological  peculiarities  of  the  muscles  innervated  by 
the  facial,  which  to  a  great  extent  lack  even  a  somewhat  fixed  point  of  inser- 
tion and  true  antagonists,  may  explain  the  exceptional  position  they  occupy 
in  this  relation. 

The  contracture  precisely  reverses,  in  the  later  stages,  the  clinical  picture 
of  grave  facial  palsy.  The  wrinkles  and  creases  on  the  affected  half  of  the 
face,  which  were  formerly  smoothed  out,  become  more  and  more  clearly 
defined,  and  become  even  deeper  than  those  on  the  healthy  side  of  the  face. 
The  palpebral  fissure  becomes  narrower,  the  corner  of  the  mouth  is  lifted 
9 


i3o  DISEASES  OF  THE  PERIPHERAL  NERVES 

up,  the  mouth  as  a  whole  is  distorted  towards  the  affected  side.  Seeing  a 
patient  in  this  stage  of  the  disease,  one  is  inclined  to  regard  the  healthy  side 
as  the  paretic  one.     Of  course,  the  functional  test  at  once  enlightens  one. 

The  contracture  is  quite  regularly  attended  by  associated  movement 
symptoms,  which,  however,  also  occur  in  the  regenerative  stage  of  cases 
recovering  without  contractures,  associated  movements  in  different  muscles 
of  the  diseased  half  of  the  face,  which  accompany  the  involuntary  droop- 
ing of  the  eyelid,  and  by  apparently  spontaneous  twitchings,  that  pass  over 
the  face  with  lightning  rapidity  and  which  in  reality  are  probably  no  more 
than  associated  movements  due  to  this  paralytic  drooping  of  the  eyelid, 
which  may  very  easily  be  overlooked.  Quite  similar  twitchings  in  the 
morbid  area  have  been  observed  when  bringing  a  hand  close  to  the  face 
quickly,  when  tapping  it  and  when  irritating  electrically  various  places  on 
both  the  sound  and  the  affected  sides:  the  so-called  reflex  twitchings.  It 
certainly  is  not  a  matter  of  a  genuine  exaggerated  reflex.  The  newer  theory, 
according  to  which  it  is,  in  all  these  cases,  a  question  of  associated  movement 
in  closure  of  the  eyelid,  has  many  points  in  its  favor.  Also  in  voluntary 
innervation  of  the  different  parts  of  the  facialis  area,  associated  movements 
appear  in  other  parts.  In  explaining  all  these  associated  movements 
it  is  very  plausibly  held,  that  in  the  regeneration  of  the  degenerated  nerve 
the  fibres  newly  arising  from  every  part  of  the  nucleus,  did  not  all  find  their 
old  muscle  area,  but  distributed  themselves  among  various  muscle  areas, 
in  consequence  of  which  in  innervations  of  the  former,  the  latter  also  contract. 
This  would  also  explain  a  peculiar  failure  in  voluntary  motility  despite  the 
restoration  of  the  electric  irritability,  since  a  muscle  area  might  have  again 
received  conductive  and  excitable  nerve  fibres,  but  not  from  its  own  nucleus. 
Therefore  this  area  can  be  irritated  electrically  through  the  nerve  and 
associated  movements  may  also  occur  in  this  area,  when  innervating 
other  parts,  but  every  attempt  at  purposive  appropriate  innervation  will 
fail. 

The  statement  that  in  the  regenerative  stage  of  grave  facial  paralyses 
an  exceedingly  great  confusion  in  the  innervation  takes  place,  very  aptly 
expresses  the  actual  conditions  (Lipschitz). 

In  facial  paralysis  not  infrequently  relapses  occur  once  or  several  times. 
The  same  side,  or  even  the  one  which  formerly  remained  sound,  is  then 
affected. 

In  deciding  upon  the  prognosis,  which  in  the  majority  of  cases  will  be 
favorable,  besides  the  fundamental  disease  the  condition  of  the  electric 
irritability  must  be  carefully  considered.  This,  though  not  invariably, 
indicates  even  in  the  second  and  third  week  with  certainty  whether  recovery 
may  be  expected  in  a  few  weeks  or  only  after  several  months.  The  prognosis 
of  grave  paralyses  will  under  all  circumstances  be  ominously  affected  by 
the  associated  movements,  which  will  seldom  be  lacking,  and  by  contracture. 


DIAGNOSIS  OF  FACIAL  PALSY  131 

It  is  often  well  to  acquaint  the  patient  with  this  prospect  at  any  early  date. 
The  danger  of  a  relapse  is,  on  the  other  hand,  of  less  importance. 

The  diagnosis  of  facial  paralysis  is  very  easy.  No  careful  investigator 
will  be  deceived  by  natural  differences  in  the  two  sides  of  the  face,  nor,  if  he 
note  the  functional  disturbances  properly,  will  he  incur  the  danger  of  mis- 
taking the  side  affected  with  paresis  for  the  sound  one,  if  contractures  exist. 
Often  the  associated  movements  at  once  reveal  the  diseased  side. 

The  real  task  of  one  who  diagnoses  such  cases  lies,  first,  in  the  distinc- 
tion of  peripheral  nerve  disease  from  nuclear,  supranuclear  and  myopathic 
paralyses,  and  secondly,  in  determining  in  what  part  of  the  course  of  the 
nerve  the  lesion  is  situated. 

The  facial  paralyses  in  myasthenia  and  in  the  different  forms  of  muscle 
dystrophy  have  quite  characteristic  peculiarities,  to  which  reference  is  made 
in  the  chapters  on  these  diseases.  As  a  rule  they  are  strictly  bilateral.  The 
reaction  of  degeneration  (R.  D.)  is  never  present.  The  myasthenic  or 
dystrophic  symptoms  in  other  areas  facilitate  the  diagnosis. 

As  a  rule,  though  not  always,  in  the  supra-nuclear  facial  paralysis 
which,  by  the  way,  is  usually  connected  with  hemiplegia,  the  upper  facial 
region,  as  a  result  of  its  bilateral  cerebral  innervation,  remains  more  or  less 
unaffected.  In  cases  where  the  paralysed  side  takes  part  in  a  normal  way 
in  the  emotionally  released  involuntary  expression,  as  well  as  in  those,  where, 
on  the  other  hand,  the  facialis  is  paralysed  only  for  emotional  impulses, 
there  is  clear  evidence  of  a  supranuclear  seat  of  the  lesion.  Testing  the  re- 
flex action  in  diagnosing  a  facial  palsy,  plays  a  subordinate  part.  Of  all 
the  more  significance  may  be  the  electric  examination  for  distinguishing 
supranuclear  from  peripheral  paralyses.  It  is  said  that  Bell's  phenomenon 
may  be  absent  in  a  central  facialis  paralysis.  Cases  are  on  record,  in  which 
— e.  g.,  in  diseases  of  the  pons — a  supranuclear  palsy  manifested  itself  on 
one  side,  and  a  palsy  occasioned  by  lesion  of  the  peripheral  neuron,  on  the 
other.  A  peripheral  paralysis  may  follow  after  a  central  one  on  the  same 
side,  if  a  focal  lesion  extends  secondarily  to  the  nucleus  or  the  nerve  roots. 

It  is  sometimes  very  difficult  or  even  impossible  to  distinguish  nuclear 
and  fascicular  cases  by  themselves  from  basal-nerve  diseases.  The  distur- 
bances in  the  secretion  of  tears,  peculiar  to  basal  palsies,  appears  to  be  lacking 
in  cases  due  to  central  disease.  Usually,  nuclear  palsies  are  accompanied 
by  other  pathognomonic  signs  of  disease  of  the  central  organ,  or  they  appear 
in  such  characteristic  forms,  that  there  can  be  no  doubt  about  a  nuclear 
disease,  as  in  the  case  of  a  bulbar  paralysis,  which  takes  a  highly  chronic, 
slowly  progressing  course,  affecting  at  first,  only  a  few  muscles  fibre  by  fibre. 
But  even  the  congenital  palsies,  which  probably  depend  on  aplasia  of  the 
nucleus  (infantile  nuclear  atrophy)  may  be  confined  to  parts  of  the  facialis 
area. 

For  a  finer  diagnosis  of  the  place  of  the  lesion  in  the  area  of  a  peripheral 


i3 2  DISEASES  OF  TEE  PERIPHERAL  NERVES 

nerve,  the  disturbances  of  taste  and  the  secretion  of  tears  are  decisive.  If 
the  lachrymal  secretion  alone  is  affected,  the  lesion  is  situated  basally,  at  all 
events  above  the  geniculate  ganglion.  Whenever  the  focus  is  situated  in 
the  area  from  which  the  large  superficial  petrosal  takes  its  origin,  disturbances 
of  lachrymal  secretion  and  of  taste  will  co-exist.  Disease  situated  below 
the  geniculate  ganglion  as  far  as  the  point  of  origin  of  the  chorda  occasions 
only  taste  disturbances.  Distally  from  the  point  of  origin  of  the  chorda, 
disturbances  both  of  taste  and  of  the  lachrymal  secretion  are  lacking.  In 
this  area  the  paralysis  frequently  affects  only  single  nerve-branches. 

It  should  be  remembered  that  the  auditory  nerve  down  to  the  geniculate 
ganglion  takes  its  course  in  close  proximity  to  the  facial.  Synchronous 
disease  of  the  auditory  nerve  may  therefore  be  significant  for  the  topical 
diagnosis  of  the  facial  paralysis. 

It  is  evident  from  the  above  that  in  inveterate  cases  the  diagnosis  will  be 
rendered  more  difficult  by  the  early  disappearance  of  the  taste  disturbance. 
The  same  is  true  of  disturbances  in  the  lachrymal  secretion. 

Therapeutics. — In  palsy  of  the  facial  nerve  the  possibility  of  treating 
the  cause  of  the  disease  has  to  be  considered  first  of  all.  In  basal  syphilitic 
meningitis,  treatment  by  inunction  promises  success.  In  ear  diseases  an 
aurist  should  be  consulted.  In  very  recent  "rheumatic"  paralyses,  some 
experienced  physicians  recommend  that  one  or  more  diaphoretic  procedures 
be  instituted. 

Furthermore,  in  the  very  first  rank  stands  electro-therapeutics,  applied 
according  to  the  general  rules  for  all  such  treatments,  but  especially  the 
stabile  cathodic  galvanization  of  the  nerve,  and  afterwards  the  irritant  appli- 
cations must  be  considered.  Systematic  exercises  aided  by  the  hand  are  to 
be  recommended. 

The  peculiar  disturbances  in  the  regenerative  stage  of  grave  cases,  as 
contracture  and  associated  movements,  place  on  the  physician  a  difficult 
and  peculiar  task.  It  is  an  altogether  erroneous  impression  that  the  con- 
tract are  is  caused  by  the  electric  treatment.  Nor,  to  be  frank,  does  it  pre- 
vent its  occurrence.  As  soon  as  the  first  symptoms  appear,  careful  massage, 
with  special  regard  to  suitable  manipulations  for  extension  and  flexibility 
should  be  instituted.  Needless  to  say,  this  treatment  should  be  undertaken 
only  by  a  professional.  To  stretch  the  cheek  and  keep  it  extended,  older 
doctors  gave  the  patient  a  wooden  ball  to  carry  under  it.  The  use  of  elec- 
tricity on  the  sound  half  of  the  face  is  quite  futile. 

To  counteract  the  associated  movements,  the  patient  should  be  taught 
to  practise  innervational  exercises  in  front  of  a  mirror. 
,     The  lagophthalmos  of  the  more  serious  kind  should  be  kept  in  mind 
from  the  start.     If  necessary,  a  protective  eye  bandage  should  be  prescribed. 

If  an  absolute  paralysis  is  persisting  in  a  case,  and  there  is  no  longer 
any  hope  of  amelioration — this  will  not  be  the  case  after  a  few  months — 


TREATMENT  OF  FACIAL  PALSY.     THE  AUDITORY  NERVE  133 

grafting  of  the  nerve  should  be  resorted  to.  According  to  the  rather  scanty 
and  not  particularly  encouraging  reports,  joining  the  peripheral  part  of 
the  facial  nerve  with  the  central  end  of  the  hypoglossus,  which  has  been 
entirely  divided  for  this  purpose,  can  be  recommended  in  preference  to  any 
other  method.  The  unilateral  palsy  of  the  hypoglossus  which  is  naturally 
produced  thereby  is  less  troublesome  to  the  patient,  and  by  a  complete  divi- 
sion, instead  of  a  mere  freshening,  the  vexatious  associated  movements 
between  the  two  areas,  which  would  otherwise  set  in  after  regeneration,  are 
avoided.  One  should  not  hope  for  too  much  from  this  operation.  For  more 
detailed  information  cf.  Lipschitz,  Monatsschrift  f.  Psychiatrie  and  Neur., 
vol.  xx,  Ergaenzungsheft,  page  84,  and  Bernhardt,  Mitteil  aus  den  Grenz- 
gebieten,  vol.  xvi,  page  476. 

In  the  residual  stage,  plastic  operations  have  now  and  then  brought  about 
a  cosmetic  and  functional  betterment.  Lagophthalmos  may  necessitate  a 
tarsorrhaphy. 

6.  The  Auditory  Xerve 

The  greater  part  of  what  we  know  concerning  diseases  of  the  auditory 
nerve,  we  owe  to  aurists.  The  following  exposition  will  show  how  impor- 
tant it  is  that  this  nerve  should  not  be  neglected,  as  is  often  the  case,  in  neuro- 
logical examinations. 

The  auditory  nerve  becomes  diseased  either  independently,  primarily, 
or  is  affected  secondarily  in  diseases  of  the  structures  that  surround  it. 

The  primary  diseases  are  tumors,  simple  atrophic  or  degenerative-neu- 
ritic  processes.  Among  the  so-called  tumors  of  the  cerebello -pontile  angle  a 
great  number  emanate  from  the  auditory  nerve  itself.  The  auditory  nerve 
atrophies  in  tabes,  where  it  is  even  an  early  symptom,  and  in  senility 
(presbyacusia) .  Primary  neuritis,  comparable  to  neuritis  optica,  is  observed 
especially  in  infectious  and  toxic  processes.  Among  poisonings  those  from 
quinine  and  salicylic  acid,  alcohol  and  tobacco  deserve  to  be  mentioned. 
Practically  more  important  are  perhaps  those  cases  of  neuritis  acustica  which 
are  caused,  as  has  been  experimentally  demonstrated,  by  acoustic  trauma 
or  by  the  long-continued  effect  of  loud  noises  (professional  deafness  of 
smiths,  gunners).  Other  causes  are  very  rare  (thunderclap),  or  entirely 
problematic  ("rheumatic"  origin).  Among  the  paralyses  of  the  cranial 
nerves  accompanying  herpes  zoster,  a  supposedly  neuritic  disease  of  the 
auditory  nerve  also  occurs. 

Secondary  disease  of  the  acoustic  nerve  we  find  in  affections  at  the  base 
of  the  brain,  particularly  in  basal  syphilitic  meningitis,  in  disease  of  the 
temporal  bone  (caries)  and  especially  in  that  of  the  labyrinth.  Slight 
labyrinthine  irritation  accompanies  various  diseases  of  the  middle  and  even 
of  the  outer  ear.  Suppurative  infections  of  the  labyrinth  occur  in  connection 
with  meningitis  (during  cerebro-spinal  meningitis,  after  measles  and  scarlet 


i34  DISEASES  OF  THE  PERIPHERAL  NERVES 

fever).  Frequently  they  occur  during  infancy  and  later  become  the  cause 
of  deaf-mutism.  Besides  this,  the  labyrinth  may  be  purulently  infected 
from  the  middle  ear.  Traumatic  injuries  of  the  labyrinth  are  usually  the 
result  of  indirect  traumas  (fracture  of  the  skull) .  When  caisson  workers 
suddenly  leave  the  place  in  which  there  is  an  increased  air  pressure,  hemor- 
rhage and  gas-embolisms  may  result.  Spontaneous  hemorrhages  have  been 
observed  in  persons  of  hemorrhagic  diathesis.  In  leucaemia,  hemorrhage 
and  leucocytic  infiltrations  occur.  In  affections  of  the  heart  the  possibility 
of  an  embolus  in  the  labyrinthine  artery  will  have  to  be  borne  in  mind. 
Frequently  syphilis,  especially  late  hereditary  syphilis,  is  the  cause  of 
labyrinthine  disease.  Concerning  the  so-called  choked  labyrinth  (corres- 
ponding to  the  choked  disk)  scarcely  anything  is  known. 

The  exact  situation  and  anatomic  nature  of  the  pathologic  changes 
which  occur  sometimes  in  the  auditory  nerve  after  mumps  are  still  in  doubt. 

Clinical  Picture. — The  auditory  nerve  is  affected  alone  or  in  associa- 
tion with  other  cranial  nerves.  The  lesion  affects  either  both  branches  or 
only  one,  either  the  vestibular  or  cochlear  nerve.  Symptoms  of  irritation 
and  of  paralysis  often  appear  simultaneously. 

Diseases  of  the  cochlear  nerve  lead  to  nerve  deafness,  the  diagnostic  sepa- 
ration of  which  from  sound  conduction  deafness,  occasioned  by  diseases 
of  the  middle  ear,  particularly  if  both  forms  be  combined,  is  not  always  easy 
and  frequently  requires  the  aurist's  utmost  skill.  The  following  symptoms 
are  characteristic  of  disease  of  the  auditory  nerve.  Complete  deafness  of 
one  ear  never  occurs  without  disease  of  the  nerve  itself.  It  is  a  peculiar 
fact  that  partial  disturbances  of  hearing  affect  especially  the  high  tones, 
that  the  limit  of  acoustic  perception  is  "narrowed  from  above,"  while  the 
deepest  tones  may  be  perceived  normally — an  occurrence  which  never  takes 
place  in  disturbances  of  sound  conduction.  In  other  cases  peculiar  "tone- 
gaps"  are  produced,  certain  parts  of  the  tone  range  are  lacking,  which  is 
also  important  in  regard  to  differential  diagnosis.  The  symptoms  of  acous- 
tic irritation  are  manifold;  somewhat  characteristic  are  those  taking  place 
in  the  area  of  high  tones,  as  the  distressingly  high  ringing  and  buzzing  in  the 
ear.  From  tests  with  the  tuning  fork,  which  is  held  now  in  front  of  the  ear, 
now  on  the  skull,  the  following  results  are  obtained:  The  duration  of  the 
perception  in  conduction  by  means  of  the  skull  is  shorter  when  contrasted 
with  the  normal,  but  never,  except  in  a  preponderating  complicated  disease 
of  the  middle  ear,  lengthened  (Schwabach's  test).  The  relation  between 
the  length  of  perception  by  air  and  by  bone  conduction  remains  normal 
and  the  tuning  fork,  which  is  heard  no  longer  by  bone  conduction,  is  still 
able  to  excite  an  auditory  impulse  if  quickly  held  in  front  of  the  ear  (positive 
effect  of  Rinne's  test) .  The  tuning  fork  applied  to  the  crown  will  be  heard 
especially  in  the  sound  ear  (lateralization  towards  the  healthy  side  in  the 
otherwise  rather  unreliable  Weber's  test). 


DIAGNOSIS  OF  DISEASES  OF  THE  AUDITORY  NERVE       135 

Diseases  of  the  vestibular  nerve  lead  particularly  to  disturbances  of  the 
equilibration  of  the  body. 

When  one  vestibular  apparatus  is  acutely  destroyed,  say  by  hemorrhage 
into  the  labyrinth,  a  sudden  attack  of  vertigo  supervenes,  in  which  the 
patient  is  prostrated,  vomits,  sometimes  faints,  usually  is  deafened  more  or 
less  completely  in  one  ear  at  the  same  time,  in  consequence  of  the  participa- 
tion of  the  cochlear  nerve,  and  is  tormented  by  violent  buzzings  (Meniere's 
symptom  complex).  The  patient  has  a  sense  of  being  twirled  in  a  swing 
or  turned  round  and  round.  He  often  complains  about  apparent  movements 
of  the  objects  he  sees.  Nystagmus  towards  the  healthy  side  exists,  caused 
or  increased  by  directing  the  look  that  way.  When,  after  some  time,  the 
patient  tries  to  stand  up  he  falls  towards  the  affected  side. 

After  a  few  days  his  condition  is  improved.  The  complaint  usually 
disappears  entirely  after  a  longer  time.  The  defect  becomes  latent,  even  a 
bilateral  loss  in  the  vestibular  apparatus  is  gradually  adjusted  through  func- 
tional compensation,  and  thereby  becomes  latent. 

In  chronic  diseases  repeated  brief  attacks  of  Meniere's  symptom  complex 
may  occur;  the  attacks  are  usually  to  be  considered  a  symptom  of  irritation. 
The  nystagmus  is  generally  directed  towards  the  affected  side.  A  pro- 
nounced inclination  to  fall  towards  a  certain  side  is  rarely  present.  In 
other  cases  a  more  or  less  continuous  feeling  of  dizziness  is  experienced, 
which  only  at  times  leads  to  a  real  attack  of  vertigo  and  in  still  others  the 
symptoms  may  vary  considerably.  The  attacks  are  then  rudimentary  and 
perhaps  only  slight,  rather  subjective  than  objective,  fits  of  giddiness  may 
manifest  themselves. 

When  chronic  disease  has  led  to  the  gradual  atrophy  of  the  vestibular 
nerve  apparatus,  the  complaints  will  disappear  (stage  of  latent  vestibular 
defect) . 

The  diagnosis  of  disease  of  the  vestibular  nerve  may,  as  is  evident  from 
the  above,  be  difficult  not  only  in  the  latent  stage  but  also  in  some  chronic 
cases.  Though  the  fully  developed  seizure  is  typical,  nevertheless  it  is 
sometimes  extremely  difficult  to  define  the  attacks  and  complaints  in  the 
rudimentary  stage.  Recently  different  methods  for  systematically  testing 
the  functional  capacity  of  the  vestibular  nerve  have  been  devised.  The 
test  for  caloric  nystagmus  appears  to  be  especially  valuable.  When  syring- 
ing an  ear  with  warm  or  cold  water,  in  normal  functioning  of  the  vestibular 
nerve,  nystagmus  in  the  same  or  in  the  opposite  direction  takes  place.  By 
means  of  this  procedure  and  other  similar  ones,  it  is  apparently  possible  to 
diagnose  both  destructive  and  irritative  conditions  of  the  nerve  with  much 
more  certainty  now  than  heretofore.  The  test  depends  upon  the  absence 
of,  or  the  ease  with  which,  caloric  nystagmus  can  be  produced.  For  more 
information  concerning  these  methods,  a  fuller  study  of  which  is  recom- 
mended, see  Barany,  Physiologie  und  Pathologie  des  Bogengangapparats, 


136  DISEASES  OF  THE  PERIPHERAL  NERVES 

Leipsic  and  Vienna,  1907,  and  Politzer,  Lehrbuch  des  Ohrenheilkunde,  5 
edition,  Stuttgart,  1908. 

In  disease  of  the  auditory  nerve  the  cochlear  and  vestibular  nerves  are  not 
always  affected  in  the  same  degree.  The  primary  diseases,  with  the  excep- 
tion of  tumors,  frequently  confine  themselves  particularly  to  the  cochlear 
nerve  and  usually  appear  bilaterally.  The  majority  of  the  secondary  diseases, 
especially  the  labyrinthine  affections,  affect,  as  a  rule,  both  the  cochlear 
and  vestibular  nerves,  but  generally  appear  only  unilaterally.  What  is 
known  as  mumps  deafness,  is,  for  the  most  part,  unilateral  and  is  unaccom- 
panied by  any  symptoms  from  the  vestibular  area. 

The  prognosis  in  most  forms  of  neuritis  acustica  is  not  unfavorable, 
but  in  almost  all  other  conditions  rather  gloomy  quoad  restitutionem. 
Many  diseases  dependent  upon  acquired  syphilis  can  be  cured  with  specific 
treatment. 

When  disturbances  of  the  apparatus  for  the  perception  of  sound  and  of 
the  vestibular  nervous  apparatus  are  found,  one  must  consider  with  refer- 
ence to  differential  diagnosis,  besides  the  diseases  of  the  peripheral  nerves, 
also  those  of  the  intra-cerebral  continuations  of  the  auditor}'-  and  vestibular 
pathways.  An  uncomplicated  and  complete  clinical  picture  of  an  attack  of 
Meniere's  disease  does  indeed  indicate  unconditionally  a  peripheral  affection. 
Otherwise,  for  a  more  minute  topical  diagnosis  one  must  ask  oneself,  whether 
the  accompanying  symptoms  point  to  the  petrous  portion  of  the  temporal 
bone,  to  the  base,  the  pons,  or  the  cerebellum,  as  the  seat  of  disease. 

It  is  to  be  hoped  that  new  and  definite  symptoms  will  be  found  in  the 
future,  by  means  of  the  new  diagnostic  method  presented  above,  for  the 
differentiation  of  peripheral  and  central  lesions  of  the  vestibular  pathways. 

The  cochlear  nerve  of  each  side  is,  in  the  brain,  immediately  connected 
with  the  bilateral  central  auditory  pathway,  so  that,  excepting  pontile  focal 
lesions,  which  affect  the  zone  of  the  root  entrance  directly,  central  auditory 
disturbances  are  always  bilateral  in  origin.  Experience  would  seem  to 
show  that  only  bilateral  focal  lesions  can  cause  any  serious  auditory 
disturbances. 

In  closing,  let  us  once  more  emphasize  how  desirable  it  is,  that  in  future 
the  disturbances  caused  by  the  eighth  cranial  nerve  be  carefully  regarded 
even  in  neurological  examinations.  Even  today  they  play  a  decisive  r61e 
in  the  diagnosis  of  the  tumors  of  the  cerebello-pontile  angle.  They  may  be 
of  value  in  an  early  diagnosis  of  tabes,  and  in  examinations  of  the  victims 
of  accidents  one  will  sometimes  by  this  routine  be  enabled  to  decide,  that 
besides  any  functional  nervous  consequences,  organic  injuries  as  well  may 
have  been  caused  by  the  trauma. 

As  to  the  treatment,  the  neurologist  should  avail  himself,  as  far  as 
possible,  of  the  services  of  an  aurist;  after  that  he  should  proceed  according 
to  general  principles.     Especially  to  be  mentioned  is  the  frequent  beneficial 


THE  GLOSSO-PHARYNGEAL  AND  VAGUS  NERVES  137 

effect  of  the  stabile  anodic  galvanization  in  cases  of  sensory  irritation.     The 
button-shaped,  active  electrode  (10  qcm.)  is  placed  on  the  tragus. 

7.  The  Glosso -pharyngeal  Nerve 

Concerning  the  diseases  of  the  glosso-pharyngeal,  but  little  definite  is 
known.  Isolated  paralyses  of  this  nerve  have  been  scarcely  ever  observed. 
Up  to  the  present  time  it  has  only  been  found  affected  in  multiple  basal- 
cranial  nerve  disease. 

Almost  invariably  the  lingual  branches  contain  the  taste  fibres  for  the 
posterior  third  of  the  tongue.  But  not  in  every  case  do  these  fibres  reach 
the  brain  in  the  glosso-pharyngeal  trunk,  for  they  may  leave  it  near  the 
petrous  ganglion  together  with  Jacobson's  nerve  and  course  to  the  trigeminal 
with  which  they  then  take  their  further  course  to  the  brain.  On  the  other 
hand,  however,  it  must  be  assumed  that  in  some  instances  the  taste  fibres 
of  the  chorda  for  the  anterior  two-thirds  of  the  tongue  are  conveyed  from  the 
facial  trunk,  not,  as  is  usual,  to  the  trigeminal,  but  to  the  glosso-pharyngeal 
by  means  of  an  anastomosis.  Finally  the  possibility  of  the  glosso-pharyngeal 
innervating,  in  exceptional  cases,  one  whole  side  of  the  tongue  can  not  be 
lightly  disregarded.  (Cf.  Fig.  28,  and  the  statements  made  concerning 
taste  innervation  in  connection  with  the  discussion  of  trigeminal  and  facial 
palsy.)  The  relation  of  this  nerve  to  the  function  of  taste  is  therefore 
undoubtedly  inconstant. 

For  the  parotid  gland  the  glosso-pharyngeal  possesses  secretory  fibres. 
They  come  into  the  gland  by  way  of  Jacobson's  nerve,  the  otic  ganglion  and 
its  anastomosis  with  the  third  trigeminal  branch.  Observations  of  aurists 
have  revealed  disturbances  in  the  functioning  of  the  parotid  gland  in  lesion 
of  Jacobson's  nerve  inside  the  tympanic  cavity. 

Unquestionably  the  glosso-pharyngeal  participates  in  the  sensory  inner- 
vation of  the  pharynx  and  supplies  also  a  part  of  the  pharyngeal  and  palatal 
musculature  with  motor  fibres.  We  have  no  definite  knowledge  with  regard 
to  the  degree  of  this  participation. 

8.  The  Vagus  (Pneumo-gastric)  Nerve 

^Etiology  of  paralysis  of  the  vagus  and  its  branches.  Basal  diseases 
usually  injure  the  vagus  together  with  its  immediate  neighbors,  especially 
the  spinal  accessory,  glosso-pharyngeal  and  hypo-glossal  nerves.  In  the 
area  of  the  jugular  foramen,  its  gate  of  exit  from  inside  the  skull,  it  anas- 
tomoses with  the  accessory  which  lies  closest  to  it.  It  takes  up  the  internal 
branch  of  this  nerve,  and  thereby  receives  a  supply  of  motor  fibres,  which 
may  be  vagus  fibres  in  reality  since,  according  to  several  investigators,  they 
emanate  from  the  vagus  nucleus.  We  have  no  certain  knowledge  regarding 
the  function  of  these  fibres. 


138  DISEASES  OF  THE  PERIPHERAL  NERVES 

Isolated  affections  of  the  vagus  always  indicate  a  lesion  outside  the 
cranium. 

In  the  neck,  gunshot  wounds  or  those  due  to  sharp  instruments,  opera- 
tive injuries,  lesions  through  pressure  by  glands,  malignant  tumors  and 
carotid  aneurisms,  may  all  lead  to  palsies  of  the  nerve  trunk.  But  also  the 
single  branches,  especially  the  important  recurrent  nerve,  may  become 
diseased  in  this  area  either  uni-  or  bi-laterally,  e.  g.,  through  tumors  of  the 
thyroid  gland  and  new  growths  in  the  esophagus. 

There  are,  besides,  intrathoracic  affections  which  cause  paralysis  of  the 
vagus,  especially  of  the  recurrent  nerve.  The  left  recurrent  winds  itself 
around  the  arch  of  the  aorta;  the  right,  from  the  front  backwards,  around 
the  subclavian  artery.  Hence  the  frequent  injury  particularly  of  the  left 
nerve  in  aortic  aneurisms  will  be  clear.  Left  sided  recurrent  palsy,  in  steno- 
sis of  the  mitral  valve,  can  usually  be  explained  by  the  effect  of  pressure 
of  the  greatly  distended  left  auricle. 

Mediastinal  tumors,  both  primary  and  metastatic,  pleuritic  and  peri- 
carditic  conditions  and  chronic-pneumonic  affections  may  injure  the  vagus 
or  recurrent  by  direct  destruction,  or  by  pressure  or  cicatricial  contractions. 

Lastly,  there  are  cases  of  affection  of  the  vagus  for  which  no  local  cause 
can  definitely  be  assigned.  Most  of  these  cases  depend  upon  toxic  or  infec- 
tious processes.  Most  frequently  we  find  palsy  in  the  innervation  area  of 
the  vagus  after  diphtheria,  at  times  as  a  partial  symptom  of  polyneuritis, 
and  at  times  isolated.  Nor  can  it  be  denied  that  sometimes  direct  injury 
of  the  nerve  through  the  diphtheritic  inflammation,  perhaps  a  kind  of  ascend- 
ing neuritis,  occurs  (Cf.  diphtheritic  paralysis  in  chapter  on  polyneuritis). 

To  what  extent  vagus  symptoms  occurring  in  tabes  depend  upon  per- 
ipheral nerve  disease,  is  doubtful. 

The  symptomatology  is  studied  best  from  the  point  of  view  of  disease 
of  the  single  branches  of  the  nerve  which  are  most  frequently  affected  alone. 
Symptoms  of  lost  function  are  the  most  important  and  in  disease  of  the  main 
trunk  usually  stand  in  the  foreground.  Paralysis  of  one  recurrent  nerve 
produces  motor  paralysis  of  one  vocal  cord.  In  cases  of  slight  lesions  the 
paralysis  is  at  first  almost  always  restricted  to  the  dilator  of  the  glottis,  the 
crico-arytenoideus  posticus  muscle  (posticus  paralysis).  The  vocal  cord  is 
adducted  into  a  position  of  phonation.  Inspiratory  abduction  is  lacking, 
while  the  closure  of  the  glottis  on  phonation  remains  unimpaired.  In  total 
paralysis  the  vocal  cord  stands  half  abducted,  in  a  medial  position,  the  so- 
called  cadaveric-position,  and  is  entirely  immobile.  The  arytenoid  cartilage 
seems  shifted  somewhat  to  the  front;  the  vocal  cord  somewhat  shortened  and 
flaccid.  In  phonation  the  sound  vocal  cord  may  approach  the  paralyzed 
One  across  the  middle  line,  so  that  a  fairly  normal  sound  can  be  produced. 
These  are  the  two  clinical  pictures  occurring  in  paralysis  of  the  recurrent. 
Other  paralyses  of  single  vocal-cord  muscles,  such  as  internus  paralysis, 


SYMPTOMATOLOGY  OF  DISEASES  OF  THE  VAGUS  139 

paralysis  of  the  interarytenoidei,  are  not  a  part  of  the  picture  of  vagus  paraly- 
sis, but  occur  chiefly  in  grave  catarrhs  of  the  larynx.  The  participation  of 
the  recurrent  in  the  sensory  innervation  of  the  larynx  is  a  question  which 
has  not  yet  been  definitely  decided. 

Vocal-cord  paralysis  in  disease  of  the  vagus  trunk  above  the  point  where 
the  recurrent  becomes  separated,  is  always,  as  has  been  said,  an  important 
partial  symptom  of  the  clinical  picture.  If,  in  cases  in  which  the  seat  of  the 
lesion  is  higher,  the  fibres  which  leave  the  vagus  in  the  superior  laryngeal, 
enter  the  area  of  the  injury,  an  anaesthesia  of  the  larynx,  and  a  paralysis  of 
the  crico-thyroid  muscle,  which  is  implicated  in  the  tension  of  the  cord,  will 
result,  though  the  loss  does  not  produce  any  clinical  symptoms  of  impor- 
tance. It  may  be  that  the  motions  of  the  epiglottis  depend  mainly  on  this 
branch  of  the  vagus.  In  very  high  seated  paralyses  of  the  vagus,  paralysis 
of  the  soft  palate  and  the  pharynx,  whose  innervation  is  accomplished 
chiefly  by  the  vagus,  may  be  expected  on  the  corresponding  side.  The 
sensory  fibres  of  the  pharynx  also  take  their  course  with  the  branches  of 
the  vagus  that  leave  the  nerve  trunk  at  about  the  level  of  the  first  cervical 
vertebra.  The  trouble  to  the  patient  in  unilateral  paralysis  is  comparatively 
slight. 

The  vagus  also  participates  in  the  innervation  of  the  heart.  The  cardiac 
branches  partly  leave  the  nerve  trunk  in  the  neck  between  the  superior  laryn- 
geal and  recurrent,  and  partly  are  branches  of  the  latter.  Finally,  the  rela- 
tion of  the  vagus  to  the  trachea,  lungs,  esophagus,  stomach,  liver  and 
other  abdominal  organs  must  be  mentioned. 

The  symptoms  of  the  whole  intestinal  innervation  area  are,  as  a  rule, 
particularly  ill  defined  and  very  difficult  to  interpret  in  diseases  of  one  nerve 
alone.  Only  when  other  and  certain  symptoms  of  a  vagus  paralysis  manifest 
themselves,  will  it  be  possible  to  connect  such  symptoms  as  bradycardia, 
tachycardia,  nausea,  etc.,  with  any  degree  of  probability  with  disturbances 
of  the  vagus. 

Bilateral  vagus  paralysis  under  any  condition  is  most  dangerous.  Very 
threatening  symptoms  are  exhibited  even  in  bilateral  posticus  paralysis. 
Severe  dyspnoea,  particularly  inspiratory  dyspnoea,  is  the  result  of  the  bi- 
lateral functional  loss  of  the  abductors  of  the  vocal  cord.  In  grave  cases, 
tracheotomy  is  necessary.  In  bilateral  simultaneous  paralysis  of  the  sup- 
erior laryngeal  and  recurrent  nerves,  the  complete  immobility  of  the  larynx 
musculature,  particularly  that  of  the  epiglottis,  the  inability  to  cough, 
thereby  resulting,  and  the  anaesthesia  of  the  larynx,  deprive  the  lungs  of 
one  of  its  most  important  protective  mechanisms,  and  it  will  rarely  be 
long  before  deadly  aspiration  pneumonia,  the  so-called  vagus  pneumonia, 
sets  in. 

Lastly,  bilateral  paralysis  of  the  pharyngeal  branches,  whose  one-sided 
functional  loss  gives  rise  to  comparatively  trifling  complaints,  leads  to  the 


i4o  DISEASES  OF  THE  PERIPHERAL  NERVES 

unfortunate  condition  of  total  paralysis  of  deglutition,  which  necessitates 
artificial  nourishment. 

Only  a  few  words  have  to  be  added  concerning  diagnosis.  A  one  sided 
recurrent,  or  still  more,  a  posticus  paralysis  may  to  the  inexperienced  be 
simulated  by  tubercular  or  carcinomatous  infiltrations  of  the  larynx,  that 
mechanically  impair  the  mobility  of  the  vocal  cord.  In  the  differential 
diagnosis  of  bilateral  posticus  paralysis,  spasm  of  the  glottis  must  be  con- 
sidered, which,  however,  occurs  only  in  transitory  attacks,  almost  exclu- 
sively in  a  very  characteristic  way  in  rhachitic  children,  and  in  rare 
instances  in  hysteria  of  adults. 

A  particularly  important  phase  of  the  diagnosis  of  vagus  paralysis  is, 
as  is  evident  from  the  astiological  discussion,  the  search  for  the  fundamental 
cause  of  the  affection. 

It  should  be  remembered  that  a  number  of  diseases  of  the  central  nervous 
system,  of  which  we  shall  name  only  bulbar  paralysis  and  focal  affections 
of  the  medulla  oblongata,  may,  by  destruction  of  the  root  and  nucleus  areas, 
even  by  bilateral  destruction  of  supranuclear  paths  (pseudo-bulbar  paraly- 
sis), lead  to  paralytic  symptoms  in  the  innervation  area  of  the  vagus. 

9.  The  Accessory  Nerve 

Isolated  paralyses  of  the  accessory  result  only,  as  in  the  case  of  the 
vagus,  through  lesions  outside  the  cranium.  The  lesion  therefore  is  always 
distal  to  the  anastomosis  of  the  two  nerves.  The  physiological  significance 
of  the  anastomosis  can  not  be  estimated  with  certainty.  In  lesions  common 
to  the  last  cranial  nerves,  carious  and  other  processes  in  the  region  of  the 
upper  cervical  vertebrae  must  be  borne  in  mind  above  all  others. 

In  isolated  accessory  paralysis  then,  it  is  a  matter  of  paralysis  of  the  so- 
called  external  branch,  which  supplies  the  trapezius  and  sterno-cleido- 
mastoid  muscles  with  motor  fibres.  The  usual  causes  are  injuries  of  the 
nerve,  particularly  those  resulting  from  extirpation  of  tubercular  glands 
of  the  neck,  and  inflammation  and  tumor  formation  in  the  immediate 
neighborhood. 

In  paralysis  of  the  sterno-cleido-mastoid  muscle,  the  head  can  be  turned 
but  feebly  towards  the  sound  side.  When  an  attempt  is  made  to  effect  this 
movement  against  resistance,  it  will  be  plainly  seen  that  the  muscle  does 
not  contract.  It  does  not  stand  out  sharply  from  the  neck  as  in  healthy 
persons.  If  a  one  sided  paralysis  exists  for  some  time  permanent  shortening 
of  the  normal  muscle  occurs,  and  a  caput  obstipum  (torticollis)  usually 
develops. 

Corresponding  to  the  physiological  function  of  the  trapezius,  which 
both  lifts  the  shoulder  girdle  and  draws  it  backwards  at  the  same  time,  the 
shoulder,  in  total  paralysis  of  this  muscle,  sinks  forwards  and  downwards. 


THE  ACCESSORY  NERVE 


141 


The  medial  edge  of  the  scapula  is  moved  outwards  from  the  spine  and  runs 
obliquely  from  the  inside  below  to  the  outside  above  (the  angel  wing  position 
of  the  scapula).  The  clavicle  springs  abnormally  forwards.  Lifting 
(shrugging)  of  the  shoulders,  and  adduction  of  the  scapula  to  the  spine,  is 
possible  only  to  a  very  limited  extent.  Through  the  lack  of  sufficient 
fixation  of  the  shoulders,  the  functional  value  of  the  arm-movements  is 
partially  impaired.  The  shoulder  girdle  is  abnormally  motile  on  passive 
movement  (Figs.  32  and  33). 

The  changes  of  the  external  form  are  still  more  strikingly  evident  in  the 
atrophic  stage.  The  neck-shoulder  line,  which  is  lengthened  on  the  affected 
side,  becomes  angular.  The  acromion  and  clavicle,  in  the  neighborhood 
of  their  articulation,  appear  to  the  touch  to  lie  under  the  skin,  as  if  they  were 
part  of  a  skeleton.  The  relief  normal  to  the  neck  is,  on  the  side  of  the 
atrophic  sterno-cleido-mastoid  muscle,  flattened,  even  depressed. 


Fig.  32. — Right-side  paralysis  of 
the  accessory.  Posterior  view. 
{Leipsic  Medical  Clinic.) 


Fig.  33. — Left-side  paralysis 
of  the  accessory.  Lateral  view. 
{Leipsic  Medical  Clinic?) 


Paralysis  of  the  trapezius  is  very  often  incomplete,  and  usually  some 
of  its  parts  remain  more  or  less  capable  of  discharging  their  functions. 
Under  such  conditions  not  only  should  incomplete  lesions  of  the  accessory 
trunk  be  thought  of,  but  also  the  fact  that  branches  of  a  few  spinal  nerves 
participate  in  the  motor  innervation  of  the  muscle  and  probably  in  an  indi- 
vidually varying  degree.  On  the  other  hand,  in  total  paralysis,  the  pos- 
sibility of  spinal  nerve  branches  destined  for  the  trapezius  muscle  becoming 
paralyzed  together  with  the  accessory,  must  be  considered.  This  occurs 
perhaps  chiefly  in  cases  in  which  the  seat  of  the  lesion  is  rather  deep. 


142  DISEASES  OF  THE  PERIPHERAL  NERVES 

The  lesion  may  be  confined  to  the  terminal  branch  destined  for  the 
trapezius,  so  that  the  sterno-cleido-mastoid  remains  intact. 

The  complication  of  disturbances  in  the  innervation  of  the  palate,  phar- 
ynx and  larynx,  indicates  intracranial  disease  in  the  region  in  which  the 
accessory  takes  its  course  in  close  proximity  to  the  vagus,  with  which  it  anas- 
tomoses through  its  internal  branch.  To  what  extent  this  branch  partici- 
pates in  causing  the  disturbances  mentioned,  cannot  yet,  as  we  have  before 
stated,  be  determined  with  certainty. 

In  the  differential  diagnosis  a  supranuclear  paralysis  will  never  occasion 
any  doubt,  as  it  invariably  appears  only  as  a  partial  symptom  of  a  cerebral 
hemiplegia. 

Nuclear  paralyses  are  found  in  progressive  spinal  muscle  atrophy  and  in 
other  diseases  of  the  spinal  cord  and  the  medulla  oblongata.  The  rare  cases 
occurring  as  symptoms  or  complications  of  tabes  are  probably  partly  nuclear, 
partly  neuritic. 

In  myopathies,  particularly  in  the  various  forms  of  muscle  dystrophy, 
the  trapezius  is  especially  often  involved;  while  the  sterno-cleido-mastoid 
atrophies  regularly  only  in  advanced  cases  of  myotonic  dystrophy. 

Congenital  muscle  defects  also  occur  in  the  area  of  the  accessory. 
In  considering  treatment  it  should  be  borne  in  mind  that  disturbances 
of  function  may  be  diminished  by  means  of  orthopaedic  apparatus  which 
gives  support  to  the  lax  shoulder  girdle. 

10.  The  Hypoglossal  Nerve 

What  has  been  said  of  the  preceding  cranial  nerves,  applies  likewise  to 
the  hypoglossal  nerve:  basal  intra-cranial  processes  and  affections  in  the 
area  of  the  upper  cervical  vertebras  paralyze  the  hypoglossal  nerve  together 
with  its  neighbors,  while  the  cause  of  isolated  paralyses  must  be  sought  out- 
side the  cranium.  Injuries  in  the  neck  are  more  likely  to  lead  to  a  paralysis 
of  the  hypoglossal  nerve,  but  palsy  now  and  then  is  occasioned  by  tumors 
or  inflammation  in  the  vicinity  of  the  nerve  trunk. 

Unilateral  paralysis  of  the  tongue  which  is  the  most  important  result 
of  disease  of  this  nerve,  causes  the  patient  but  slight  trouble  and  disturbance. 
The  tongue  can  be  moved  only  imperfectly  towards  the  sound  side.  As  a 
rule  it  deviates  towards  the  affected  side  when  extended,  which  is  usually 
the  case  when  the  genio-glossus  muscle  is  involved  in  the  paralysis — a  con- 
dition which  will  hardly  be  absent  in  peripheral  nerve  diseases.  When 
resting  in  the  mouth,  the  tongue  does  not  usually  show  any  considerable 
deviation  from  the  normal  position. 

On  the  other  hand,  the  signs  of  atrophy  will  soon  be  distinctly  visible, 
namely,  the  decrease  in  its  volume,  the  wrinkling,  the  flaccid,  spongy  consis- 
tency of  half  of  the  tongue  and  further  the  reaction  of  degeneration.    Remark- 


THE  HYPOGLOSSAL  NERVE  143 

ably  often  fascicular  and  fibrillary  spasmodic  twitchings  of  the  diseased 
half  and  even  of  the  whole  tongue  have  been  reported  in  cases  of  paralysis 
of  the  tongue  which  were  surely  not  nuclear. 

The  hypoglossal  nerve  innervates  also  the  so-called  infra-hyoid  muscles 
(sterno-hyoid,  thyro-hyoid,  sterno-thyroid,  omo-hyoid).  It  receives  the 
fibres  for  these  muscles  in  part  through  several  anastomoses  with  the 
superior  cervical  nerves.  When  the  lesion  is  comparatively  deep  seated, 
those  fibres,  which  reach  the  nerve  only  peripherally,  may  in  part  be  affected 
also.  In  such  cases  the  symptoms  of  the  paralysis  of  the  muscles  concerned 
are  said  to  be  more  pronounced  and  the  hyoid  bone  and  larynx  deviate 
when  swallowing  towards  the  sound  side;  it  is  further  claimed  that  a  flat- 
tening of  the  neck  in  the  muscle  area  is  observable  and  that  R.  D.  may  be 
detected. 

Bilateral  paralysis  of  the  tongue  which,  it  is  true,  hardly  ever  results 
from  peripheral  causes,  is  of  course  much  more  serious.  Eating  and 
speaking  are  much  hindered  in  such  cases. 

In  the  differential  diagnosis,  in  addition  to  disease  of  the  motor  nerve 
of  the  tongue,  supranuclear  glossoplegia  must  be  first  considered.  It  occurs 
unilaterally  as  a  partial  symptom  of  cerebral  hemiplegia  and  only  in  ex- 
tremely rare  cases  as  an  isolated  monoplegia.  The  symptoms  indicative  of 
the  peripheral  neuron  are  of  course  lacking.  Bilateral  supranuclear  glos- 
soplegias  belong  to  the  picture  of  pseudobulbar  paralysis.  Nuclear  paral- 
yses are  found  in  bulbar  paralysis  and  in  all  sorts  of  other  diseases  of  the 
medulla  oblongata.  The  paralysis  is  then  very  frequently  a  partial  one; 
single  muscles,  as  the  important  genioglossus,  may  remain  unaffected; 
functional  disturbances  may  be  almost  wholly  wanting,  and  the  disease  may 
be  disclosed  only  through  circumscribed  atrophy  accompanied  by  fibrillary 
tremor.  Tabetic  hemiatrophy  of  the  tongue  is  probably  nuclear  in  some 
cases,  neuritic  in  others.  The  radicular  paralyses,  too,  may  be  partial,  in 
explanation  of  which  it  is  necessary  only  to  consider  the  relatively  great 
extension  of  the  root-area. 

Except  in  myasthenia,  myopathic  paralyses  are  very  rare.  Congenital 
unilateral  defects  have  been  described  and  also  the  participation  of  the 
tongue  in  hemiatrophia  faciei. 

Glossospasm,  which  occurs  unilaterally,  will  not  be  mistaken  by  any 
careful  investigator  for  a  unilateral  glossoplegia.  The  rigid  fixation  of 
the  tongue  in  the  spasm  prevents  such  an  error. 

B.  The  Spinal  Nerves 

In  the  following  discussion  of  the  diseases  of  the  spinal  nerves,  only 
those  cases — selected  from  an  abundance  of  possibilities — will  be  treated  in 
detail  which  are  typical    and  of   practical   importance.     Those  who  are 


144  DISEASES  OF  THE  PERIPHERAL  NERVES 

familiar  with  the  fundaments  of  the  science  of  peripheral  paralyses  and 
know  the  innervation  areas  of  the  various  nerves,  will  be  able  to  judge  cor- 
rectly even  rarer  clinical  pictures. 

i.  The  Phrenic  Nerve 

The  phrenic  nerve  may  be  injured  in  the  neck,  or  in  the  interior  of  the 
thorax  by  injuries,  tumors  and  inflammations. 

The  paralyses  of  the  diaphragm  resulting  from  the  influence  of  toxic 
and  infectious  factors  without  apparent  local  cause,  are  as  a  rule  bilateral 
and  probably  appear  most  frequently  in  polyneuritis.  Diseases  of  this 
nerve  are  rare.  For  the  purposes  of  differential  diagnosis  it  may  be  well, 
however,  to  enumerate  the  other  causes  of  paralysis  of  the  diaphragm:  the 
most  varied  diseases  of  the  spinal  cord  in  the  area  of  the  third  and  fourth 
cervical  roots  and  the  disease  of  these  roots  themselves  in  morbid  processes 
of  the  meninges  or  of  the  vertebrae.  Tabes  in  a  few  rare  cases  leads  to 
diaphragmatic  paralysis,  which  sometimes  at  least  is  of  an  undoubtedly 
spinal  nuclear  nature.  Further  there  are  myopathic  diaphragmatic  paral- 
yses; the  progressive  muscular  dystrophy  may  seize  upon  the  diaphragm. 
Inflammations  of  the  serous  membrane,  which  covers  the  diaphragm,  may 
spread  to  the  muscle  and  occasion  a  myositic  paralysis.  In  hysteria  a  picture 
of  a  complete  suspension  of  the  diaphragmatic  functions  may  be  observed. 
Finally,  for  the  sake  of  completeness,  the  congenital  defects  which  occur  in 
the  diaphragm  in  the  peculiar  form  of  hernia  diaphragmatica  should  be 
mentioned.     This  latter  needs  no  consideration  in  differential  diagnosis. 

Symptoms. — In  inspiration  the  paralyzed  diaphragm  either  does  not 
descend  at  all  or  does  so  very  incompletely.  For  this  reason  the  inspiratory 
arching  of  the  upper  abdomen  is  lacking  and  it  is  even  possible  that  during 
deep  inspiration  these  regions  may  be  drawn  in.  In  paresis,  the  diaphragm 
is  capable  of  a  moderate  though  sufficient  bulging;  but  its  descent  may  be 
prevented  through  light  pressure  with  the  hands  against  the  abdominal 
walls.  Litten's  diaphragm  phenomenon  is  absent  in  the  paralyses.  The 
edge  of  the  lung  may  be  shifted  somewhat  upwards.  An  X-ray  examination 
will  show  that  the  diaphragm  stands  high,  that  it  does  not  bulge  normally 
during  inspiration  and  that,  during  deep  inspirations,  it  will  sometimes 
even  be  drawn  upwards. 

In  unilateral  paralysis  the  symptoms  are  well  defined  only  on  one  side, 
while  the  subjective  troubles  and  functional  disturbances  may  be  relatively 
trifling.  Bilateral  paralysis,  on  the  other  hand,  must  be  regarded  as  quite 
a  serious  occurrence.  Even  when  at  rest  the  patient  finds  breathing 
somewhat  difficult.  The  slightest  extra  demands  on  him  occasion  great 
anxiety.  Much  will  depend  upon  the  functional  capacity  and  practice  in 
costal  breathing.  A  supervening  disease  of  the  lungs  produces  grave 
dangers. 


THE  PHRENIC  NERVE.     THE  BRACHIAL  PLEXUS 


145 


The  abdominal  pressure  is  very  much  impaired  in  its  influence  on  the 
viscera  in  the  abdominal  cavity  through  the  lack  of  resistance  from  above. 
The  abdomen,  when  pressed,  does  not  arch.  The  force  of  coughing  and 
sneezing  is  also  diminished  on  account  of  impairment  of  the  functional 
capacity  of  the  abdominal  muscles  involved  in  paralysis  of  the  diaphragm. 

Nothing  special  need  be  added  concerning  the  prognosis  and 
therapeutics. 

The  physician  should  be  familiar  with  electric  irritation  of  the  phrenic 
nerve  because  by  this  means  in  some  cases  of  asphyxia,  for  instance  in 
narcotic  poisoning,  the  respiration  and  consequently  the  life  of  the  patient 
can  be  kept  up  for  several  hours  and  time  thus  be  gained  for  saving  the 
patient. 

2.  The  Brachial  Plexus  and  its  Nerves 

The  network  of  the  arm  nerves  consists  chiefly  of  the  four  lower  cervical 
nerves  and  the  first  dorsal  nerve.  The  scheme  in  Fig.  34  shows,  how  from 
these  five  roots  emanate  first  three  plexus  cords  and  from  these  again, 
through  renewed  reunion  and  the  splitting  off  of  fibres,  three  secondary 


Plexus  roots 

Primary 

^jLtiZsT  Plexus  cord 


Secondary 
Plexus  cord 


Ntnusculocu  taneus 

N.inedianup 

M  radial,  u.  axillar 

N.  uhiaris 


>  tftfwracicuf    LonffUS 
Fig.  34. — Schematic  presentation  of  the  plexus  brachialis. 


cords,  one  lateral,  one  medial,  and  one  posterior.  From  the  portion  of  it 
situated  above  the  clavicle,  the  plexus  furnishes  quite  a  number  of  its  "short" 
branches  to  the  musculature  of  the  shoulder.  Below  the  clavicle,  in  the 
axilla,  the  long  nerves  of  the  arm  emanate  from  the  secondary  cords,  the 
posterior  of  which  sends  out  the  short  axillary  nerve  also  (Fig.  34). 

As  a  supplement  to  the  schematic  representation,  which,  as  far  as  pos- 
sible, should  be  kept  handy  for  reference,  the  following  must  be  added. 
Of  the  short  supraclavicular  branches  only  a  few  have  been  represented. 
The  subscapular  nerve  should  be  mentioned,  which  usually  emanates 
together  with  several  roots,  from  the  branches  of  the  primary  bundle, 
that  unite  in  the  secondary  posterior  cord.  From  the  medial  (lower) 
plexus  cord  the  medial  cutaneous  branches  of  the  arm  and  forearm 
usually  originate. 


146  DISEASES  OF  THE  PERIPHERAL  XERVES 

Although  in  the  main  the  type  described  is  regularly  found,  yet  the 
structure  of  the  plexus  occasionally  shows  quite  considerable  individual 
variations.  Thus,  the  fasciculus  secondarius  posterior,  instead  of  arising 
from  the  primary  cords,  may  originate  from  the  spinal  nerves  themselves, 
of  which  it  is  constituted.  The  manner  and  place  of  separation  of  the  various 
short  branches  are  likewise  liable  to  numerous  variations.  On  that  account 
it  would  be  somewhat  hazardous  to  go  into  minute  details  in  a  topical  diag- 
nosis of  paralysis  of  this  area. 

We  distinguish  root  paralyses,  true  plexus  paralyses,  and  paralyses  of 
the  peripheral  nerres  in  the  more  restricted  sense  of  the  term. 

It  is  a  case  of  root  paralysis  if  the  spinal  nerves,  designated  above  as 
roots  of  the  plexus,  suffer  a  lesion  themselves,  before  forming  the  primary 
plexus  bundle.  A  distinction  can  be  made  between  intravertebral  and 
extravertebral  root  paralyses.  The  most  frequent  causes  are  morbid  pro- 
cesses in  the  area  of  the  meninges  or  the  vertebrae,  traumas  influencing  the 
spinal  cord  directly  or  its  immediate  vicinity,  such  as  knife  cuts  and  localized 
tumor-like  structures.  In  unilateral  paralysis  the  symptoms  of  lost  function 
will  correspond  to  the  innervation  area  of  the  affected  root.  This  will  be 
treated  of  with  the  spinal  cord  diseases. 

Pluriradicular  paralyses  cannot,  for  the  reasons  just  stated,  be  dis- 
tinguished so  easily  from  the  corresponding  plexus  paralyses,  unless  some 
astiological  factors  or  some  concomitant  symptoms  of  the  disease  enable  a 
more  definite  local  diagnosis. 

Broadly  speaking  three  main  forms  may  be  distinguished  among  the 
plexus  paralyses:  first,  the  superior  plexus  or  Duchenne-Erb  paralysis; 
secondly,  the  inferior  plexus  or  Klumpke's  paralysis;  and  thirdly,  total  plexus 
paralysis. 

^Etiology. — Mechanical  injury  to  the  plexus  stands  pre-eminent  among 
all  the  original  causes.     It  is  produced  by  either  direct  or  indirect  influences. 

The  direct  lesions  usually  occur  in  the  supraclavicular  fossa  or  the 
axilla.  Contusions,  stabs  or  gunshot  injuries  may  affect  the  plexus;  in 
serious  trauma  of  the  shoulder  it  may  be  affected  by  bone  fragments  or 
hemorrhage.  The  plexus  may  also  be  endangered  by  tumors,  or  even  be 
destroyed  by  them;  in  the  armpits  it  may  be  paralyzed  by  the  constant 
pressure  of  crutches. 

In  indirect  mechanical  influences  it  is  usually  a  case  of  traction,  some- 
times perhaps  of  a  contusion  occasioned  by  the  clavicle.  We  shall  not 
enter  into  a  detailed  discussion  of  the  much  disputed  mechanism  of  the 
latter  occurrence.  It  becomes  particularly  dangerous  when  the  arm  is 
drawn  well  down,  outwards,  while  the  head  is  at  the  same  time  inclined  in 
the  opposite  direction.  It  is  easy  to  see  that  in  this  way  traction  is  made 
upon  the  plexus.  But  it  is  sometimes  the  result  of  lifting  the  arm  strongly 
up  and  backwards  and  letting  it  remain  some  time  in  this  position.     In 


THE  SPINAL  NERVES  147 

both  these  ways  plexus  paralyses  result,  as  for  instance,  in  some  exercises 
on  a  horizontal  bar  and  in  breaking  in  wild  horses. 

Dislocations  of  the  humerus  as  well  as  brusque  attempts  to  replace  it 
are  also  significant,  since  especially  in  the  latter  case  a  direct  lesion  of  the 
plexus  may  occur. 

It  is  probable  that  in  plexus  paralysis  of  porters  or  longshoremen  the 
direct  pressure  of  the  load  exerts  an  influence  besides  the  traction  upon  the 
plexus. 

The  cervical  ribs  are  mentioned  as  playing  a  predisposing  role. 

A  special  position  is  occupied  by  the  so-called  obstetric  paralysis.  With 
infantile  obstetric  paralysis  we  have  already  become  acquainted  when 
discussing  the  facial  form.  Much  more  important  and  frequent  are  the 
plexus  paralyses  of  the  newly  born,  which  occur  intrapartum,  and  almost 
exclusively  in  deliveries  by  artificial  means.  The  specific  reasons  are  the 
same  as  in  those  cases  of  paralysis  acquired  in  later  life:  direct  pressure 
on  the  plexus,  whether  produced  by  fingers  or  instruments,  more  serious 
complicating  lesions  of  the  plexus,  when  a  fracture  of  the  clavicle  or  humerus 
has  taken  place,  traction  in  unfortunate  positions  of  the  arms,  manual 
traction  upon  an  arm  or  awkardly  releasing  an  uplifted  arm. 

Lastly,  the  so-called  narcotic  paralyses  must  be  mentioned  which  may 
affect  either  single  arm  nerves  or  the  plexus  itself.  And  in  these  cases,  too, 
there  is  no  special  mechanism  through  which  they  originate.  The  foremost 
cause  of  these  unpleasant  occurrences  is  an  unsuitable  position  of  the  arms 
for  some  time  during  a  lengthy  narcosis.  The  feeble  or  non-resistance  of  the 
patient  by  giving  rise  to  a  favorable  opportunity  for  a  traumatic  attack  upon 
the  plexus,  at  all  events  plays  a  greater  role  than  the  acute  toxic  process 
which  has  been  assumed  by  some  to  be  the  principal  cause  of  the  paralysis. 

Plexus  paralyses  founded  on  generally  injurious,  toxic-infectious  influ- 
ences, without  any  visible  local  cause,  are  extremely  rare.  In  many  cases 
of  so-called  plexus  neuritis  which  have  been  observed  in  infectious  diseases 
of  the  lungs  it  may  be.  that  the  main  cause  was  a  neighboring  injury  from 
an  upper  lobe  and  not  a  general  toxic  process. 

By  far  the  most  frequent  form  of  plexus  paralysis  is  the  Duchenne-Erb 
"upper"  plexus  paralysis.  It  is  usually  brought  on  by  direct  mechanical 
causes,  attacking  the  supra-clavicular  fossa  and  by  the  indirect  mechanical 
influences  enumerated  before.  To  this  group  also  belong  most  of  the 
obstetric  paralyses.  The  uppermost  primary  plexus  trunk  is  affected. 
The  auxiliary  branch  of  the  secondary  posterior  bundle  emerging  from  the 
two  uppermost  roots  is  regularly  affected.  The  deltoid,  biceps,  brachialis, 
brachio-radialis  (supinator  longus)  muscles  and  frequently  also  the  supinator 
(brevis)1  muscle  are  paralyzed.     The    suprascapular   nerve   is  very  often 

1  Hence  the  muscles  regularly  affected  are  those  that  in  a  normal  condition  may  be  irritated 
together  electrically  from  Erb's  point  in  the  supraclavicular  fossa. 


148  DISEASES  OF  THE  PERIPHERAL  NERVES 

caught  in  the  area  of  the  lesion,  in  which  case  the  supra-  and  infraspinatus 
are  likewise  paralyzed.  The  subscapularis  is  less  often  affected.  It  can 
be  no  surprise  to  any  one  who  is  conversant  with  the  topographic  anatomy 
to  find  that  other  complications  may  arise.  But  in  such  instances  it  is  no 
longer  a  typical  occurrence,  but  a  more  or  less  rare  variation  of  the  disease. 
The  symptoms  of  lost  function  are  as  follows:  The  ability  in  the  shoulder 
joint  to  lift  the  arm  is  partially  or  wholly  destroyed.  Unless  the  mus- 
culature of  the  forearm,  particularly  that  flexor  group  emanating  from  the 
condylus  internus  humeri,  in  time  vicariously  assumes,  as  it  frequently  does, 
a  part  of  the  function,  it  is  impossible  to  bend  the  elbow.  The  defect  of  the 
brachio-radialis  muscle  will  become  most  obvious  when  the  patient  tries  to 
bend  the  forearm,  with  it  occupying  a  median  position  between  pronation 
and  supination,  against  resistance.  The  muscle  does  not  project  in  such 
cases,  as  it  does  under  normal  conditions,  from  the  forearm  on  the  side  of  the 
radius,  forming  a  characteristic  edge,  but  on  the  contrary  remains  flat  or 
perhaps  it  even  sinks  in,  while  the  entire  forearm  seems  "cylindrical." 
Supination  of  the  flexed  forearm  (biceps)  is  impossible;  supination  of  the 
forearm  in  extension  becomes  possible  only  in  case  of  aid  from  the  supinator 
brevis.  Quite  frequently  the  patient  is  unable,  in  the  shoulder  joint,  to 
rotate  the  arm  outwards  (when  the  suprascapularis  is  affected).  The 
position  alone  of  a  small  child's  arm  may  often  lead  to  a  conjecture  of  the 
nature  of  the  disturbance;  for  the  arm  is  then  adducted,  the  elbow  in 
extension,  the  forearm  in  pronation  and  perhaps  the  entire  arm  turned 
inwards. 

Disturbances  of  sensibility  occur  in  the  innervation  area  of  the  fifth  and 
sixth  cervical  roots  which  forms  a  broad  stripe,  descending  on  the  outer  side 
of  the  arm  and  forearm. 

Klumpke's  "inferior"  plexus  paralysis  is  much  less  frequent.  It 
affects  the  plexus  cord  which  originates  through  union  of  the  eighth  cervical 
with  the  first  thoracic  nerve.  The  most  common  causes  of  such  paralysis 
are  direct  mechanical  disturbances,  tumors,  and  trauma.  It  would  be 
difficult  to  state  how  many  of  such  cases  are,  strictly  speaking,  plexus 
paralyses  and  how  many  radicular  paralyses. 

The  paralysis  may  be  restricted  to  the  small  muscles  of  the  hand,  the 
thenar  and  hypothenar  eminences  (the  balls  of  the  thumb  and  little  finger), 
and  the  interossei.  Occasionally  a  few  muscles  of  the  flexor  group  of  the 
forearm  are  affected.  The  clinical  picture  becomes  especially  characteristic 
when  the  ramus  communicans  of  the  first  thoracic  nerve  is  drawn  into  the 
area  of  the  disturbance.  In  that  case  paralysis  of  the  dilator  pupillae  of 
the  side  concerned  results.  The  disturbances  of  sensation  occupy  a  stripe- 
like area  descending  along  the  inside  of  the  arm  and  along  the  ulnar  side 
of  the  forearm.  This  area  is  innervated  by  the  two  spinal  nerves  mentioned 
above. 


ROOT  AND  PLEXUS  PARALYSIS  149 

Total  plexus  paralysis  affects  all  branches  of  the  plexus  more  or  less 
completely.  As  the  disturbances  retrogress,  the  paralysis  may  confine 
itself  to  one  or  another  part.  In  some  cases  the  area  of  the  radial  and 
circumflex  (secondary  posterior  plexus  cord)  appears  to  be  most  seriously 
and  lastingly  injured.  If  the  lesion  has  started  in  the  axilla,  the  clinical 
picture  may  resemble  a  complete  paralysis  of  the  long  branches  of  the  arm 
nerves,  which  results,  for  instance,  from  encircling  the  arm  too  tightly. 

Even  when  otherwise  very  grave  and  extensive,  the  disturbances  of  sensi- 
bility often  leave  the  most  proximal  part  of  the  inside  of  the  upper  arm 
unimpaired.  This  part  receives  sensory  fibres  from  the  intercosto-humeral 
nerve,  which  does  not  belong  to  the  brachial  plexus. 

The  diagnosis  of  root  paralysis  and  of  plexus  paralysis  in  recent 
cases  is  not  difficult  as  a  rule,  if  a  careful  investigation  is  made  and  the 
aetiological  circumstances  are  duly  considered.  Mistaking  such  cases  for 
those  impediments  to  motion  resulting  solely  from  bone  lesions  can  always 
be  avoided.  Of  course,  spinal  diseases  situated  in  the  primary  segments 
of  the  plexus  roots  may  lead  to  symptoms  of  lost  function,  which  correspond 
more  or  less  closely  to  those  of  the  plexus  paralysis.  But  often  other  signs 
of  an  intraspinal  affection  will  manifest  themselves  in  such  cases.  Serious, 
even  insurmountable,  difficulties  may  be  found  in  distinguishing  it  from  a 
poliomyelitis  that  is  restricted  to  an  arm  area,  though  probably  only  in  such 
inveterate  cases  in  which  no  information  can  be  secured,  concerning  the 
conditions  under  which  the  disease  developed.  The  absence  of  disturbances 
of  sensation,  which  latter  almost  without  exception  in  poliomyelitis  remains 
normal  from  the  very  beginning,  does  not  afford  a  differentiating  criterion 
in  inveterate  cases.  Judging  residual  conditions  is  made  particularly 
difficult  by  the  fact  that  after  partial  recovery  the  defects  may  confine 
themselves  irregularly  to  some  single  muscles. 

It  must  not  be  forgotten,  besides,  that  both  intra-  and  antepartum  cerebral 
and  spinal  diseases  and  paralyses  of  single  peripheral  nerves  may  be  acquired 
in  some  rare  cases.  Not  every  paralysis  affecting  the  arm  of  a  newly  born 
child  is  a  plexus  paralysis. 

Statistically  considered  the  prognosis  of  a  plexus  paralysis  is  decidedly 
less  favorable  than  that  of  a  single  peripheral  nerve  paralysis.  Perhaps 
this  is  due  to  the  fact  that  the  anatomic  injuries  are  much  more  serious  than 
is  usually  supposed.  For  this  reason  an  early,  careful  and  active  treatment, 
and  a  vigilant  observance  of  the  course  of  the  disease  are  urgently  demanded 
in  every  case.  Especially  in  these  cases  is  any  negligence,  and  particularly 
a  "laisser  aller"  particularly  reprehensible.  It  is  self  evident  that  compli- 
cating bone  lesions  require  surgical  treatment.  Even  the  nerve  injury  itself 
may  necessitate  an  operation.  If  there  was  no  occasion  in  the  early  stages 
for  suturing  the  nerve,  nevertheless,  if  the  neurological  treatment  remains 
without  results  for  some  time,  one  should  consider  the  feasibility  of  finding 


150  DISEASES  OF  THE  PERIPHERAL  NERVES 

the  plexus  secondarily,  and  if  needed,  paving  the  way  for  the  restoration  of 
function  by  excision  of  scars  and  by  suture.  The  results  obtained  by  such 
treatment  have  been  encouraging. 

It  is  well  to  state,  that  especially  on  account  of  the  peculiar  irritation 
conditions  obtaining  in  the  first  few  weeks  after  birth,  electric  prognosis  in 
affections  of  the  newly  born  does  not  as  a  rule  give  reliable  results. 

A  knowledge  of  the  origin  of  plexus  paralyses  should  induce  the  surgeon 
and  the  obstetrician  to  use  whatever  prophylactic  care  may  be  necessary. 

The  Single  Peripheral  Nerves   That  Emerge  from  the  Brachial 

Plexus 

a.  Short  Branches  Originating  Above  the  Clavicle 

The  only  paralysis  in  this  group  of  any  practical  significance  is  that  of  the 
long  thoracic  nerve,  because  it  alone  appears  more  frequently  independently. 
Its  causes  are  similar  to  those  of  the  plexus  paralysis :  mechanical  influences 
in  the  supraclavicular  region  and  in  the  axilla,  certain  large,  straining,  long 
continued  movements,  in  connection  with  traction  upon  the  arm,  especially 
raising  the  shoulder  joint.  In  some  cases  generally  injurious,  toxic  infectious 
influences  occupy  a  prominent  place. 

The  clinical  symptom  of  this  paralysis  is  the  paralysis  of  the  large 
serratus  anticus  muscle.  As  a  result  of  the  preponderant  pull  of  its  antago- 
nist, the  shoulder  blade,  when  at  rest,  usually  stands  somewhat  higher  and  its 
inner  edge  is  nearer  to  the  spinal  column  than  on  the  sound  side.  The  in- 
ferior angle  of  the  shoulder  blade  usually  stands  off  a  little  from  the  thorax, 
and  thereby  slips  out  from  the  covering  formed  by  the  edge  of  the  latissimus 
dorsi.  The  ability  to  raise  the  arm  sideways  and  especially  forwards  is 
seriously  impaired;  in  fact,  the  arm  as  a  rule  cannot  be  lifted  higher  than  a 
horizontal  position.  In  time,  however,  a  far  reaching  compensation  may 
take  place,  in  which  numerous  muscles,  among  which  the  supraspinatus  and 
trapezius  muscles  stand  first,  participate.  In  all  cases,  however,  the  serratus 
does  not  contract  when  the  arm  is  raised,  which  is  revealed  by  inspection  and 
palpation  of  its  indentations.  The  characteristic  turning  of  the  shoulder 
blade  which  moves  its  inferior  angle  outwards  and  upwards,  and  which, 
occasioned  by  the  serratus,  sets  in  as  a  rule  not  when  the  arm  is  beginning  to 
be  lifted,  but  shortly  before  it  reaches  a  horizontal  position,  is  likewise 
lacking.  Insteadjof  this,  the  above  mentioned  anomalies  in  position,  which 
even  when  at  rest  are  usually  visible,  become  more  prominent  when  the  arm 
is  raised  sideways ;  in  particular  the  medial  edge  of  the  scapula  moves  close  to 
the  spinal  column.  In  addition  to  this,  in  raising  the  arm  forwards,  the 
medial  edge  stands  off  considerably  backwards  from  the  thorax,  so  that  it  is 
possible  to  feel 'the  under  surface  of  the  scapula  or  the  subscapular  muscle. 
This  position  has  been  designated  as  the  "winged"  position  of  the  scapula 
(Fig-  35)- 


PARALYSIS  OF  THE  LONG  THORACIC  NERVE 


151 


In  diagnosing  the  paralysis,  particular  dependence  must  be  placed  upon 
the  absence  of  visible  and  palpable  contractions  of  the  muscle  and  also  upon 
the  absence  of  the  characteristic  movement  of  the  scapula  when  it  is  attempted 
to  raise  the  arm.  An  over-estimation  of  the  anomalies  in  position  has  led  to 
the  adoption  of  a  pseudo-serratus  paralysis.  In  some  very  thin  persons  a 
kind  of  "wing"  position  of  the  scapula  may  be  observed,  which  in  asymmet- 
rically built  people  may  appear  even  unilaterally,  when  no  serratus  paralysis 
exists.  In  such  cases  it  is  very  easy  to  prove  that  the  muscle  is  capable  of 
performing  its  functions. 


Fig.  35. — Right-side  paralysis  of  the  serratus.     Elevation  of  both  arms  laterally  and 
slightly  forwards.     {After  Curschmann-Schiiffner,Leipsic  Medical  Clinic!) 

For  persons  engaged  in  manual  labor  a  serratus  paralysis  is  a  serious  de- 
fect, but  the  prognosis,  in  which  one  should  consider  the  possibility  of  com- 
pensation, is  not  on  the  whole  unfavorable. 

The  other  supraclavicular  branches  of  the  plexus  are,  indeed,  seen  in 
plexus  paralyses  some  more  and  some  less  often  affected,  but  their  isolated 
paralyses  are  neurological  rarities,  which  are  observed  now  and  then  espe- 
cially after  mechanical  injuries  of  the  primary  area  of  these  nerves.  Defects 
in  the  area  of  the  muscles  innervated  by  them  are  usually  caused  not  by  a 
disease  of  the  nerves,  but  by  myopathic-dystrophic,  and  above  all,  by  arthritic 
processes  (arthritic  atrophy  of  the  supra-  and  infraspinatus),  and  central 


152  DISEASES  OF  THE  PERIPHERAL  NERVES 

affections.  Congenital  defects,  particularly  in  the  pectoral  area,  may  also 
occur.  In  the  latter  cases  the  compensation  is  often  remarkable  for  its 
completeness. 

b.  Branches  Developing  From  the  Infraclavicular  Plexus  Section 

Circumflex  Paralysis. — ^Etiological  factors  are  traumatic  influences  in 
the  shoulder  and  supraclavicular  areas,  pressure  acting  in  the  axilla,  traction 
upon  the  nerve  occasioned  by  excessive  raising  of  the  arm  and  by  dislocation 
of  the  shoulder-joint,  and  in  some  cases  by  noxious  factors  of  a  toxic  and  in- 
fectious nature.  In  those  cases  in  which  the  paralysis  results  from  sleeping 
upon  the  uplifted  arm,  as  well  as  in  infantile  obstetric  paralysis  and  that 
produced  during  narcosis,  pulling  or  stretching  the  nerve  is  the  chief  cause. 

The  clinical  symptoms  are  paralysis  of  the  deltoid  muscle  and  anaesthesia 
in  the  cutaneous  area  supplied  by  the  nerve.  The  muscle  cannot  be  made  to 
contract;  neither  can  the  arm  be  raised  from  the  trunk.  The  occasional 
preservation  from  injury  of  an  anterior  muscle  segment  which  derives  its 
fibres  from  the  anterior  thoracic  nerve,  is  of  no  special  functional  value. 
On  the  other  hand,  in  the  later  stage,  a  partial  functional  compensation  may 
be  possible  by  means  of  numerous  auxiliary  muscles,  among  which  the  supra- 
spinatus,  which  is  specially  important  in  the  earlier  stages  of  the  movements, 
stands  foremost. 

The  paralysis  of  the  teres  minor,  which  is  likewise  supplied  by  the  circum- 
flex, cannot,  as  a  rule,  be  shown. 

In  regard  to  differential  diagnosis,  the  fact  is  very  important  that  the 
deltoid  muscle  exceedingly  often  undergoes  an  arthritic  atrophy,  while  its 
nerve  remains  unimpaired.  To  determine  such  cases,  the  affection  of  the 
joint  should  be  proved  first  of  all.  In  arthrogenous  atrophy,  both  the  supra- 
and  the  infraspinatus  are  regularly  affected,  R.  D.  is  absent,  and,  of  course, 
the  disturbances  of  sensation  in  the  circumflex  area  are  always  lacking. 

Further,  direct  myopathic  paralyses  of  the  deltoid  muscle  resulting  from 
blows  or  pressure  upon  the  muscle  (as  from  lying  upon  the  shoulder)  have 
been  described,  and  these,  too,  might  lead  to  a  confusion  between  the  two. 

Because  of  its  rarity,  we  shall  do  no  more  than  simply  mention  the  par- 
alysis of  the  musculo -cutaneous. 

Musculo -spiral  or  radial  paralysis  is  among  the  most  frequent 
peripheral  paralyses.  It  owes  its  special  predilection  to  the  extremely  ex- 
posed situation  of  the  nerve.  During  the  greater  part  of  its  course  along  the 
arm,  the  musculo-spinal  nerve  lies  directly  on  the  periosteum  of  the  humerus, 
and  particularly  where  it  entwines  itself  around  the  bone  on  the  external  side 
of  the  arm  is  it  exposed  to  all  kinds  of  injuries.  In  the  first  place,  bone 
fractures  in  the  most  diverse  places  may  affect  the  nerve  at  once,  as  well  as 
lead  later  to  so-called  secondary  traumatic  paralysis.    Further,  the  nerve  may 


MUSCULO-SPIRAL  PARALYSIS  153 

suffer  lesions  from  pressure  in  its  course  along  the  arm,  in  the  armpit  from 
crutches,  and  in  different  places  through  an  unfortunate  position  of  the  arm 
during  sleep  or  narcosis.  Sometimes  stretching  plays  a  part,  as  when  the 
arm  has  lain  in  an  extremely  raised  position.  Dislocation  of  the  shoulder 
joint  may  also  cause  a  radial  paralysis.  It  is  superfluous  to  enumerate  the 
many  possibilities  of  direct  lesion  by  blows,  shots,  etc.  Paralysis  by  con- 
stricting cords  through  encircling  the  upper  arm,  which  affects  the  radial  in 
particular,  has  already  been  mentioned.  Now  and  then,  paralyses  are 
occasioned  by  bungling  injections  of  ether  or  other  substances,  or  by  in- 
jections in  unsuitable  places.  In  some  cases,  the  paralysis  has  been  due  to  a 
sudden  violent  contraction  of  the  triceps  muscle. 

In  individual  predispositions,  such  as  exist,  e.g.,  in  toxic  conditions  and  in 
tabetic  individuals,  frequently  very  slight,  external  local  influences  suffice  to 
occasion  the  paralysis. 

The  so-called  lead  paralysis  of  the  musculo-spiral  nerve  will  be  discussed 
with  polyneuritis. 

Clinical  Picture. — In  the  vast  majority  of  cases,  the  paralysis  affects 
only  the  group  of  extensors  of  the  forearm,  and  the  disturbance  of  sensation 
is  limited  to  the  skin  area  of  the  hand,  which  is  supplied  by  the  musculo- 
spiral  nerve.  The  motor  symptoms  of  lost  function  are  as  follows:  Exten- 
sion of  the  wrist  and  of  the  fingers — except  where  rendered  possible  by 
means  of  the  interossei  and  lumbricales  muscles — the  supination  of  the 
extended  arm  and  abduction  of  the  thumb,  in  so  far  as  not  enabled  by  the 
abductor  brevis,  become  impossible.  An  important  synergist  for  bending 
the  elbow  was  lost  in  the  brachio-radial  muscle  (supinator  longus),  whose 
defect  can  be  ascertained  best,  if,  as  has  already  been  mentioned  when 
discussing  Erb's  paralysis,  the  patient  is  made  to  flex  the  forearm,  when 
in  a  semipronated  position,  forcefully  against  resistance.  In  a  normal 
condition,  the  muscle  on  the  radial  side  of  the  forearm  will  then  project 
sharply,  while  in  a  radial  paralysis  no  contraction  whatever  occurs. 

This  loss,  however,  impairs  also  the  functional  capability  of  a  number  of 
nonparalyzed  muscles.  Doubling  the  fingers  into  a  fist  can  be  accomplished 
but  feebly,  if  the  synchronous  extension  of  the  wrist  be  wanting.  The 
ability  of  the  patient  to  spread  his  fingers  out  normally,  and  to  execute 
lateral  movements  of  the  wrist,  can  be  demonstrated  only  when  his  fingers 
and  hand  have  been  assisted  to  retain  that  semi-extended  position  favorable 
to  this  function,  but  which  they  cannot  assume  by  themselves. 

When  the  hand  is  left  to  itself,  it  hangs  down  limp  from  the  wrist,  the 
fingers  are  somewhat  flexed  and  the  thumb  is  slightly  opposed  (wrist  drop) . 

Only  comparatively  rarely,  when  the  seat  of  the  lesion  is  very  high,  does 
the  triceps  muscle  share  in  the  paralysis.  In  such  cases  the  disturbance  of 
sensation  may  also  affect  the  cutaneous  innervation  area  of  the  musculo- 
spiral  on  the  forearm  (N.  cutan.  antibrach.  dorsalis)  and  even  on  the  arm 


154  DISEASES  OF  THE  PERIPHERAL  NERVES 

'  (N.  cutan.  brach.  posterior).  The  participation  of  the  musculo-spiral  nerve 
in  the  innervation  of  the  brachialis  anticus  muscle  (outer  part)  may  under 
certain  circumstances  be  shown  conclusively  by  electric  examination. 

That  the  motor  paralysis  from  the  beginning  is  restricted  to  part  of  the 
muscles  of  the  extensor  group,  is  likewise  observed  only  comparatively 
seldom.  This  form  is  most  likely  to  occur  when  the  lesion  is  situated  on 
the  forearm. 

The  disturbances  of  sensation  in  musculo-spiral  paralysis  are  usually 
trifling. 

A  peculiar  form  of  trophic  disturbance,  manifesting  itself  as  a  thickening 
of  the  tendons  and  which  is  designated  as  Gubler's  tendon  swelling,  has  been 
observed  particularly  in  musculo-spiral  paralysis,  in  the  region  of  the  back 
of  the  hand.     It  does  not  seem  to  be  of  any  great  importance. 

The  prognosis  of  musculo-spiral  paralysis,  statistically  considered, 
is  exceedingly  favorable.  Because  of  the  exposed  position  of  the  nerve,  a 
particularly  large  number  of  cases  of  slight  paralysis  are  observed.  It  has 
been  shown  convincingly,  that  especially  in  musculo-spiral  paralysis,  early 
and  proper  electric  treatment  affects  very  favorably  the  prognosis  in  regard 
to  the  time  required  for  recovery  (E.  Remak). 

As  a  supplement  to  the  general  rule  for  the  treatment  we  add  that  in 
incurable  musculo-spiral  paralysis,  orthopedics  has  at  its  disposal,  besides 
operative  treatment,  suitable  corrective  apparatus  constructed  as  cuffs 
with  attached  gloves,  serving  to  replace  by  rubber  traction  the  loss  of  the 
extensors,  which  is  so  troublesome  in  using  the  hand. 

Median  Paralysis. — The  various  kinds  of  mechanical  injuries  which, 
as  we  have  seen,  threaten  the  musculo-spiral  especially  in  the  area  of  the 
arm,  may  all  (with  the  exception  of  a  paralysis  caused  by  sudden  muscular 
contraction)  affect  the  median  also,  though  on  account  of  the  protected  posi- 
tion of  the  nerve  this  occurs  much  less  frequently.  Among  the  most  fre- 
quent causes  of  median  paralysis  is  complete  division  of  the  nerve  by  wounds 
resulting  from  cuts  or  stabs  on  the  anterior  side  of  the  forearm.  Ascending 
neuritis  in  consequence  of  infected  wounds  in  the  area  of  the  terminal 
ramifications  has  been  observed  particularly  in  the  median. 

If  the  nerve  sustains  a  lesion  in  its  course  down  to  the  elbow  joint,  there 
ensues  a  paralysis  of  the  entire  flexor  group  in  the  forearm  with  the  exception 
of  the  muscles  supplied  by  the  ulnar,  of  the  flexor  carpi  ulnaris,  and  of  that 
part  of  the  flexor  profundus  digitorum  destined  for  the  two  last  fingers; 
further,  a  paralysis  of  the  ball  of  the  thumb  (excepting  the  adductor  pollicis 
muscle)  and  a  disturbance  of  sensation  in  the  greater  part  of  the  palm  of 
the  hand  belonging  to  the  median  nerve. 

The  alteration  of  motor  functions  is  therefore  as  follows :  Pronation  of  the 
forearm  becomes  impossible.  The  wrist  can  be  bent  feebly  only  when  aided 
by  a  simultaneous  abduction  toward  the  ulnar  side.    Flexing  the  second  and 


ULNAR  PARALYSIS  155 

third  phalanges  is  possible  only  in  the  last  two  fingers  and  even  there  only  to 
a  limited  extent.  By  means  of  the  interossei  and  lumbricales,  flexing  the 
proximal  phalanges  from  the  second  to  the  fourth  finger,  under  synchronous 
extension  of  the  other  joints,  remains  intact.  The  apposition  of  the  thumb 
disappears. 

Especially  as  a  result  of  this  disappearance  the  hand  assumes  that  peculiar 
position,  which  has  caused  it  to  be  designated  as  ape-hand. 

Generally  speaking  the  disturbances  of  sensation  are  much  more  strongly 
pronounced  in  median  paralysis  than  in  other  peripheral  paralyses  of  the 
extremities. 

If  the  median  suffers  an  injury  not  far  from  the  wrist,  which  happens  not 
infrequently,  the  clinical  symptoms  are  restricted  to  the  disturbance  of 
sensation  and  to  the  paralysis  of  the  ball  of  the  thumb — still,  however,  with 
the  exception  of  the  adductor. 

Ulnar  Paralysis. — What  has  been  said  concerning  the  mechanical 
aetiology  of  median  paralysis  is  true  also  of  the  ulnar.  It  must  be  added, 
however,  that  besides  being  exposed  to  mechanical  injuries  on  the  flexor  side 
of  the  forearm,  where  it  is  not  seldom  injured,  e.  g.,  by  cuts,  the  ulnar  is 
especially  exposed  to  them  also  in  the  region  of  the  internal  condyle  of  the 
humerus.  Above  all  pressure  paralyses  occasionally  result  from  lesions  at 
this  point,  for  instance  when  during  sleep  the  head  is  propped  up  by  the 
elbow.  A  particular  predisposing  condition  for  this  arises,  when  the  nerve 
is  not  securely  seated  in  a  deep  sulcus,  but,  being  in  a  position  of  habitual 
dislocation,  easily  slips  from  its  resting  place.  This  condition  appears  either 
congenitally  or,  in  rare  cases,  is  due  to  traumatic  influences.  Paralyses  of 
the  ulnar,  apparently  originating  spontaneously,  especially  in  persons  predis- 
posed to  them  through  toxic  influences  or  infectious  processes  are  described 
likewise  (Fig.  36,  A  and  B). 

In  injuries  located  in  the  lower  part  of  the  forearm,  the  paralysis  is 
restricted  to  the  interossei  and  lumbricales,  the  muscles  of  the  ball  of  the  little 
finger  and  to  the  adductor  pollicis.  The  pure  adduction  of  the  thumb, 
both  the  abduction  and  adduction  movements  of  the  other  fingers,  and, 
moreover  particularly  the  flexion  of  the  proximal  phalanges  of  the  second  to 
the  fifth  fingers  with  synchronous  extension  of  their  second  and  third  joints 
are  suspended.  The  last  named  function  is  usually  impaired  less  in  the 
second  and  third  fingers  than  in  the  other  two,  as  the  first  two  of  the  lum- 
bricales, which  just  in  this  movement  participate  as  synergists  of  the 
interossei,  as  a  rule,  derive  their  motor  fibres  from  the  median. 

In  high  seated  lesions  of  the  ulnar  nerve,  the  flexor  carpi  ulnaris,  which 
bends  the  wrist  towards  the  ulnar  aspect  of  the  arm,  and  the  flexor  profundus 
digitorum  for  the  last  two  fingers,  which  participates  in  the  flexion  of  their 
second  and  third  phalanges,  are  also  paralyzed.  The  sinking  in  of  the 
interosseous  spaces  and  the  atrophy  of  the  ball  of  the  little  finger  are 


156 


DISEASES  OF  THE  PERIPHERAL  NERVES 


accompanied  then  by  a  characteristic  flattening  of  the  ulnar  aspect  of  the 
forearm. 

The  typical  position  of  the  fingers,  overextension  of  the  first  phalanges 
and  flexion  of  the  others  from  the  second  to  the  fifth  fingers,  dependent 
upon  the  preponderance  of  healthy  antagonists,  is  designated  as  claw  or  talon 
hand,  main  en  griff e.  The  anomaly  is  most  strongly  pronounced  in  the  fifth 
finger,  least  in  the  second,  as  can  be  easily  understood  from  the  above 
statements. 

The  disturbances  of  sensation  occur  in  a  greater  or  smaller  part  of  the 
innervation  area  of  the  ulnar  nerve  situated  on  the  ulnar  side  of  the  hand. 
The  ulnar  edge  of  the  hand  and  the  small  finger  are  rather  regularly 
hypothetic. 


A  B 

Fig.  36. — A,  paralysis  of  the  left  ulnaris,  caused  by  the  division  of  the  nerve  in  the  forearm. 
The  normal  hand  alongside.  B,  the  affected  hand  from  the  volar  side.  Atrophy  of  the  hand- 
muscles  supplied  by  the  ulnaris,  claw  or  talon  hand.  The  formation  of  the  usually  present 
hyperextension  of  the  basal  phalanges  of  the  ring  and  little  fingers  was  prevented  in  this  case  by 
traction  from  the  scar  located  at  the  flexor  side  of  the  forearm.     (Leipsic  Medical  Clinic.) 

Ulnar  paralysis  seems  to  cause  a  certain  predisposition  to  Dupuytren's 
fascia  contraction. 

When,  in  the  general  part,  the  reader  was  cautioned  against  confusing  a 
peripheral  paralysis  with  all  kinds  of  malformations  and  impairments  of 
movements,  mechanically  produced  by  cicatricial,  arthritic,  and  other 
processes,  which  may  be  accompanied  by  muscle  atrophy  and  even  slight 
numbness  of  sensation,  it  was  really  more  particularly  the  ulnar  paralysis 
that  was  alluded  to.     Careful  examinations  will  prevent  such  errors. 

In  the  treatment  of  ulnar  paralysis,  the  habitual  dislocation  of  the  nerve 
must  be  considered  in  some  cases.  It  may  be  remedied  by  various  operative 
methods. 


THORACIC  NERVES.     LUMBAR  AND  SACRAL  PLEXUSES     157 

3.  The  Other  Thoracic  Nerves 

It  is  extremely  rare  that  paralyses  of  this  area  come  under  clinical 
observation.  Now  and  then  paralyses  of  the  abdominal  muscles  result  from 
a  disease  of  the  lower  thoracic  nerves,  chiefly  occasioned  by  pathological 
processes  that  injure  the  roots  concerned  in  the  area  of  the  spinal  meninges. 
Sometimes,  however,  they  may  appear  in  connection  with  infectious  factors 
and  herpes  zoster. 

Motor  disturbance  is  most  clearly  shown  in  the  attempt  to  exert  abdom- 
inal pressure.  The  entire  abdomen,  or  the  diseased  side,  is  spherically 
arched,  but  no  contraction  of  the  abdominal  muscles  can  be  seen  or  felt. 
It  becomes  impossible  to  raise  the  trunk  from  a  lying  position  without 
assistance  from  the  hands.  In  this  attempt,  too,  the  inability  to  exert  the 
abdominal  muscles  can  be  clearly  seen  and  felt.  In  unilateral  paralysis  the 
umbilicus,  which  even  when  at  rest  sometimes  deviates  somewhat,  is  drawn 
considerably  towards  the  sound  side.  The  abdominal  reflex  is  absent  in 
the  area  of  the  disease,  and  hypaesthesia  may  usually  be  shown  to  exist. 

Complete  paralysis  of  the  abdominal  muscles  leads  to  the  development 
of  an  abnormally  increased  lordosis  of  the  lumbar  portion  of  the  spine. 
More  serious  is  the  impossibility  of  making  strong  expiratory  movements. 

Much  more  frequent  than  the  peripheral  paralysis  of  abdominal  muscles 
is  their  participation  in  progressive  muscle  dystrophy  and  in  varied  spinal 
diseases.     Congenital  defects  also  occur. 

4.  Nerves  of  the  Lumbar  and  Sacral  Plexuses 

The  plexus  of  the  leg  (plexus  lumbo-sacralis)  arises  from  the  lumbar 
nerves  and  from  the  first,  second  and  half  of  the  third  sacral  nerve.  These 
roots  of  the  plexus  participate  in  the  formation  of  the  cauda  equina,  and 
proceed  by  a  more  or  less  extensive  course  along  the  inside  of  the  spinal 
canal,  where  diverse  pathological  processes  may  injure  them.  Radicular 
paralysis  of  this  area  resulting  from  such  injury  will  be  dealt  with  in  the 
chapter  on  spinal  diseases.  After  their  exit  from  the  intervertebral  foramina, 
the  first  three  lumbar  nerves  and  half  of  the  fourth  unite  to  form  the  trunk 
of  the  crural  nerve,  after  they  have  sent  out  a  number  of  secondary  branches : 
a  few  small  ones,  mostly  sensory,  and  the  more  important  obturator  and 
lateral  femoral  cutaneous  nerves  (external  cutaneous).  So  much  for  the 
area  of  the  lumbar  plexus.  Now,  the  other  half  of  the  fourth  lumbar  nerve, 
having  united  with  the  fifth  to  form  the  lumbo-sacral  cord,  coalesces  with 
the  sacral  nerve  segments  of  the  leg  plexus  into  the  sciatic  nerve.  The  roots 
of  this  sacral  plexus  area  send  out  several  secondary  branches,  namely,  the 
gluteal  nerves,  the  posterior  femoral  cutaneous  (perforating  cutaneous)  and 
the  motor  nerves  for  the  small  outward  rotators  of  the  thigh,  the  piriformis, 
obturator  internus,  two  gemelli  and  the  quadratus  femoris  muscles.     The 


158 


DISEASES  OF  THE  PERIPHERAL  NERVES 


Jliohypogastricus 
Jlioinguinalis 

(jenitqfemoralis 

Cutanea?  lateralis 

Cruralis 

Odturatoriujs 


(flutaeus  superior 


branch  for  the  first  mentioned  rotator  muscle  always  emanates  from  the 
roots;  the  others  may  arise  from  the  sciatic  trunk  (Fig.  37). 

Concerning  peculiar  groupings  of  symptoms,  which  would  correspond 
to  lesions  of  certain  plexus  segments  and  which  might  be  clearly  differentiated 
from  pictures  of  paralyses  of  peripheral  branches,  we  do  not  possess  the 
definite  knowledge  that  has  been  obtained  for  the  brachial  plexus.  In 
affections  of  the  leg  plexus,  we  are  usually  confronted  with  the  same  clinical 
pictures  as  in  cases  of  single  nerve  paralysis,  such  as  crural  paralysis,  sciatic 
paralysis,  etc.  Some  few  remarks,  which  will  perhaps  be  of  value  for  the 
localization  in  the  plexus  area,  will  be  given  in  the  following  paragraphs 
dealing  with  paralyses  of  the  more  important  single  nerves. 

Anterior  Crural  Paralysis. — Among  the  more  important  causes  of 
this  rare  paralysis  are  the  following:  Injury  of  the  nerve  by  direct  wounding, 
through  tumors,  particularly  those  near  the   spinal  column,  in  the  pelvis, 

also  by  means  of  the  pressure  which 
the  nerve  may  sustain  from  the 
body  of  the  child  during  protracted 
delivery  (maternal  obstetric  paral- 
ysis); pressure  injuries  caused  by 
protracted  extreme  flexion  of  the 
hips,  such  as  have  been  observed  in 
infantile  obstetric  paralysis  in  chil- 
dren born  with  breech  presentation, 
or  after  narcoses  during  which  the 
leg  was  awkwardly  placed;  lesions 
of  the  nerve  in  fracture  of  the  pel- 
vis and  in  fractures  and  dislocations 
of  the  thigh.  Lastly,  a  crural  par- 
alysis may  in  some  cases  be  ob- 
served without  any  visible  local 
cause  in  persons  who  were  under 
the  influence  of  metabolic  disturbances  or  of  infectious  and  toxic  processes. 
The  most  important  and  the  most  regularly  occurring  consequence  of  a 
crural  paralysis  is  the  paralysis  of  the  quadriceps  femoris  muscle.  Active 
extension  of  the  knee  joint  becomes  an  impossibility.  The  patient  can 
support  himself  on  the  leg  only  while  he  keeps  the  knee  in  a  passively  forced 
extended  position.  As  soon  as  it  is  flexed,  he  collapses.  For  this  reason^he 
swings  the  diseased  leg  forwards  when  walking,  and  after  he  has  put  down 
the  foot,  passively  presses  the  knee  into  extension  until  the  other  leg  again 
supports  the  body.  The  paralysis  of  the  sartorius  can  be  determined  by 
inspection  and  palpation;  it  does  not,  however,  occasion  any  considerable 
functional  disturbances.  The  paralysis  of  the  little  branch,  through  which 
the  anterior  crural  participates  in  the  innervation  of  the  pectineus,  appears 


Jschiadicup 

Glutaeus  infer to r 
Cutaneusjemorip  post. 

Fig.  37. — Scheme  of  the  lumbosacral  plexus 


PALSY  OF  THE  LATERAL  FEMORAL  CUTANEOUS  NERVE     159 

to  have  no  significance.  In  high  seated  lesions  the  ilio-psoas  is  likewise 
paralyzed.  This  results  in  a  loss  of  flexion  in  the  hip  and  walking  is  rendered 
extremely  difficult.  A  bilateral  quadriceps  paralysis  also  renders  walking 
very  uncertain,  while  in  a  bilateral,  complete  paralysis  of  the  anterior  crural 
nerve  walking  becomes  altogether  impossible. 

Besides  motor  disturbances,  sensory  ones  in  the  extended  area  of  the  skin 
supplied  by  the  anterior  crural  nerve  are  part  of  the  disease  picture.  The 
patellar  tendon  reflex  is  destroyed. 

It  is  hardly  necessary  to  emphasize  that  a  concomitant  affection  of  the 
secondary  branches  of  the  lumbar  plexus  may  sometimes  acquire  a  topical- 
diagnostic  significance.  For  parts  of  the  innervation  area  of  this  peripheral 
nerve  to  participate  in  the  most  diversified  and  different,  myopathic  and 
nerve  diseases  occurs  much  more  frequently  than  its  rare  paralysis.  One 
of  the  most  frequent  causes  of  a  weakness  resembling  paralysis  in  the  quad- 
riceps area,  is  an  arthrogenous  atrophy  of  the  muscle  in  diseases  of  the  knee 
joint. 

Therapy. — It  must  be  emphasized  that  an  orthopaedic  apparatus,  which 
replaces  the  functions  of  the  lost  muscles  by  elastic  bands,  may  materially 
improve  the  ability  to  use  the  leg. 

Obturator  paralysis  is  still  more  uncommon.  It  manifests  itself  alone 
or  accompanies  anterior  crural  paralysis.  iEtiologically  affections  in  the 
pelvic  area  must  be  mentioned.  An  obturator  hernia  may  lead  to  paralysis 
of  the  nerve;  both  maternal  obstetric  and  narcosis  paralyses  have  been 
observed. 

The  adductors  of  the  thigh,  of  which  only  the  pectineus  and  magnus  are 
slightly  innervated  from  other  sources,  are  paralyzed,  and  so  is  the  obturator 
externus,  which  participates  in  the  external  rotation  of  the  thigh.  Besides 
this,  disturbances  of  sensation  may  manifest  themselves  on  the  inside  of  the 
thigh. 

The  paralysis  of  the  lateral  femoral  cutaneous  nerve  (external 
cutaneous)  is  of  greater  practical  interest.  There  is  a  peculiar  clinical  pic- 
ture, known  as  meralgia  ■paresthetica,  Bernhardt-Roth's  disturbance  of 
sensibility,  caused  by  an  isolated  destructive  disease  of  the  lateral  femoral 
cutaneous  nerve.  Usually  it  appears  unilaterally;  patients  complain  of 
various  unpleasant  sensations  and  pains  on  the  outer  side  of  the  thigh,  which 
become  particularly  pronounced  when  walking.  All  mechanical  irritations, 
even  the  pressure  of  the  clothes,  become  very  unpleasant.  Some  patients 
even  complain  of  a  feeling  of  heaviness  in  the  calf,  manifesting  itself  when 
they  walk  and  somewhat  resembling  intermittent  claudication.  Objectively, 
a  hypaesthesia  or  anaesthesia  exists  in  a  smaller  or  greater  part  of  the  innerva- 
tion area  of  the  nerve.  The  seat  and  extension  of  the  anaesthesia  may 
fluctuate.  Very  often  pressure  will  reveal  particularly  painful  points  along 
the  course  of  the  nerve. 


160  DISEASES  OF  TEE  PERIPHERAL  NERVES 

The  aetiology  of  the  disease  is  obscure.  It  develops  in  connection  with 
infectious  diseases,  such  as  typhoid  and  articular  rheumatism.  It  has  been 
seen  to  follow  injurious  mechanical  influences  and  overexertion,  and  in 
patients  suffering  from  disordered  metabolism,  and  it  has  been  ascribed, 
more  or  less  correctly,  to  several  other  factors.  The  affection  is  of  a  harmless 
nature;  nevertheless  it  may  become  very  troublesome  to  quite  a  few  of 
those  who  suffer  from  it.  The  prognosis  is  very  questionable,  for  the 
disease  may  be  very  obstinate  and  even  relapses  may  occur. 

Of  therapeutic  measures,  faradization  may  often  be  recommended,  though 
in  cases  with  great  irritation  the  stabile  anode  galvanization,  in  which  the 
inactive  electrode  can  be  placed  on  the  calf,  is  usually  to  be  preferred.  It 
has  been  reported  that  in  some  cases  a  resection  of  the  nerve  was  successful. 

Sciatic  Paralysis.  ^Etiology. — Even  in  their  course  through  the 
pelvis  the  sciatic  fibres  may  sustain  various  injuries.  One  of  the  most  im- 
portant causes  is  maternal  obstetric  paralysis  of  the  nerve,  occasioned  by  the 
pressure  to  which  in  protracted  delivery  the  nerve  is  exposed.  Besides 
lesions  may  result  from  tumors,  inflammatory  processes  of  neighboring 
organs,  or  pelvic  exudates  from  fracture  of  the  pelvis  and  of  the  lowest  part 
of  the  spinal  column.  Traction  and  stretching  are  produced  especially  by 
dislocations  of  the  hip;  sometimes  also  by  careless  attempts  to  reduce  them. 
At  the  time  when  brusque  stretchings  of  the  nerve  were  still  used  as  a  treat- 
ment of  neuralgia,  paralyses  were  seen  to  result  from  them  as  well  as  from 
remedial  injections  into  and  below  the  gluteal  musculature. 

Further,  peripherally  the  nerve  is  especially  endangered  by  fractures 
of  the  femur. 

In  the  upper  part  of  the  popliteal  space  the  two  terminal  branches, 
the  peroneus  and  tibialis  nerves  (external  and  internal  popliteal),  separate. 
Along  their  individual  course  they  are  exposed  to  numerous  dangers,  which 
require  a  special  discussion. 

The  peroneal  nerve  (external  popliteal  nerve)  is  affected  especially,  fre- 
quently by  pressure  influences,  as  for  instance,  in  occupations  which  must  be 
carried  on  in  a  stooping,  squatting  position  (e.  g.,  workers  in  sugar  beet  fields), 
by  pressure  during  narcosis  or  sleep  and  in  particularly  predisposed  persons, 
even  from  keeping  one  leg  over  the  other  for  a  long  time.  Traction,  for 
instance  from  a  sudden  twist  in  the  ankle,  affects  in  particular  the  superficial 
branch.  Frequently  fractures  of  the  leg,  especially  of  the  fibula,  affect  the 
nerve;  but  on  the  other  hand,  affections  of  the  knee  joint,  in  spite  of  their 
proximity,  only  extremely  seldom  exert  a  harmful  influence.  The  equally 
rare  case  of  an  infantile  obstetric  paralysis  of  the  peroneus  nerve  occasioned 
by  delivery  of  the  child  by  the  foot  is  mentioned  on  account  of  its  prophylactic 
interest. 

The  tibialis  nerve  (internal  popliteal  nerve)  is  much  less  endangered. 
In  its  more  protected  position  it  suffers  less  frequently  from  pressure  lesions; 


PERONEAL  PALSY  161 

probably  the  most  frequent  cause  of  its  isolated  paralysis  is  a  direct 
trauma. 

The  nerves  of  the  sciatic  area  are  also  sometimes  found  to  be  affected 
with  an  isolated  paralysis,  though  no  local  cause  can  be  determined.  As  a 
rule,  a  toxic  or  infectious  condition  or  a  metabolic  disturbance  may  be  shown 
to  have  been  a  strongly  predisposing  factor.  Connected  with  protracted 
neuralgia  of  the  sciatic  nerve  are  often  symptoms  of  lost  function,  and  even 
more  or  less  pronounced  paralysis  symptoms. 

The  discussion  of  symptomatology  begins  most  appropriately  with  a 
discussion  of  the  symptoms  of  peroneal  and  tibial  paralysis. 

(a)  Peroneal  Paralysis. — In  complete  paralysis  the  extension  (dorsal 
flexion)  of  the  ankle  and  of  the  proximal  phalanges  of  the  toes  as  well  as  the 
pronation  (abduction)  of  the  foot  are  entirely  lost.  Extension  of  the  second 
and  third  phalanges  is  considerably  impaired  and  is  now  possible  only  with 
the  aid  of  the  interossei  and  lumbricales,  which  at  the  same  time  flex  the 
proximal  joints.  Supination  (adduction)  of  the  foot  is  seriously  impaired 
by  the  loss  of  the  synchronous  extension  of  the  anterior  tibial.  The  foot 
assumes  a  pes  equirius  position,  while  the  toes  are  flexed.  To  prevent  the 
toes  from  dragging  when  he  walks,  the  patient  has  to  lift  his  knee  to  an 
abnormal  extent  (steppage  gait1).  In  protracted  paralysis  and  with 
insufficient  medical  aid,  the  abnormal  position  of  the  foot  will  become 
permanently  fixed  by  the  contracture  of  antagonists.  Since  the  pronation 
of  the  foot  is  completely  suspended  and  yet  a  few  supinators  are  preserved 
(those  supplied  by  the  tibial  nerve),  there  is  added  to  the  pes  equinus  position 
a  slight  raising  of  the  medial  border  of  the  foot.  Consequently  a  pes  equinus 
varus  is  formed. 

Disturbance  of  sensation  affects  the  dorsal  surface  of  the  foot  and  toes 
and  a  short  adjoining  portion  outside  on  the  extensor  surface  of  the  leg. 
The  cutan.  surge  lateralis  nerve  (communicans  peronei)  which  originates 
in  the  popliteal  space  immediately  after  the  division  of  the  peroneal  and  the 
tibial  nerves,  is  not  considered  in  this  discussion  of  isolated  peroneal  paralysis. 

It  has  already  been  mentioned,  and  it  will  be  enlarged  upon  when 
discussing  sciatic  paralysis,  that  the  two  main  branches  of  the  peroneal 
nerve  do  not  always  participate  to  the  same  extent.  Even  direct  injuries 
may  affect  only  one  of  them.  The  superficial  branch  (musculo-cutaneous) 
innervates  only  the  peronei  muscles  and  but  a  very  small  part  of  the  region 
of  the  skin.  Everything  else  devolves  upon  the  deeper  branch  (anterior 
tibial  nerve).  To  understand  the  functional  losses  and  anomalies  of 
position  manifesting  themselves  in  cases  of  partial  paralyses  of  the  peroneus 
region,  it  must  be  remembered  that  in  paralysis  of  the  anterior  tibial  the 
foot  is  disposed  to  assume  a  valgus  position;  in  paralysis  of  the  peronei 

1  Charcot  compared  this  gait  to  that  of  the  grave  step  of  noble  horses;  steppeur,  Engl,  stepper, 
from  to  step,  meaning  particularly  a  dignified  step . 


1 62  DISEASES  OF  THE  PERIPHERAL  NERVES 

muscles,  on  the  other  hand,  it  tends  to  assume  a  varus  position.  The 
peroneus  longus  muscle  which  is  inserted  at  the  base  of  the  first  metatarsal 
bone,  is  also  an  antagonist  of  the  tibialis  anterior  muscle  in  so  far  as  it  lowers 
its  point  of  insertion  anteriorly  and  increases  the  arching  of  the  foot.  If 
it  is  paralyzed,  a  flattening  sets  in;  in  case  of  its  contracture  an  increase  of 
the  arching  of  the  ankle.  The  function  of  extension  devolves  solely  upon 
the  ramus  profundus. 

(b)  The  tibial  paralysis  affects  the  musculature  of  the  calf,  the  muscles 
of  the  balls  of  the  big  and  the  little  toe,  the  interossei  and  lumbricales. 
Flexion  of  the  foot  and  toes  is  lost — the  slight  flexor  action  of  the  peroneus 
longus  is  hardly  of  any  value  in  view  of  the  grave  defect — the  patient  is 
unable  to  spread  out  his  toes  or  to  adduct  them;  neither  can  he  extend  the 
second  and  the  third  toe  joints  with  a  synchronous  flexion  of  the  proximal 
phalanges.  Supination  is  possible  only  by  means  of  the  tibialis  anterior, 
therefore  only  with  simultaneous  extension.  The  foot  assumes  slight 
extension  and  pronation  (i.  e.,  calcaneo-valgus)  position;  the  toes  in  the 
metatarso-phalangeal  joint  suffer  over-extension  (claw  foot,  pied  en  griff e). 

The  disturbance  of  sensation  occurs  in  the  plantar  region  of  the  foot, 
including  the  flexor  surface  of  the  toes  and  the  outer  area  of  the  ankle, 
perhaps  even  a  short  contiguous  portion  outside  on  the  posterior  aspect  of 
the  leg. 

The  Achilles  tendon  reflex  is  destroyed.  The  plantar  reflex  is  either 
destroyed  or  at  any  rate,  if  the  sensory  conduction  is  preserved,  abnormal. 
The  characteristic  flexion  of  the  toes  is  suspended  on  account  of  an  inter- 
ruption in  the  motor  path.  The  extension  of  the  toes  which  takes  its  place, 
especially  in  the  case  of  the  big  toes,  does  not,  of  course,  have  the  diagnostic 
significance  of  the  Babinski  reflex  (pseudo-Babinski). 

(c)  Sciatic  Paralysis. — If  the  nerve  suffers  a  complete  interruption  of 
conductivity  above  the  place  where  the  separation  of  the  peroneal  and  the 
tibial  takes  place,  it  will  result  in  an  absolute  paralysis  of  all  the  muscles  in 
the  leg  proper  and  in  the  foot.  In  high  seated  lesions,  the  flexor  muscles 
of  the  thigh  (biceps,  semimembranosus  and  semitendinosus)  are  also 
paralyzed.  The  loss  of  the  little  branch  by  which  the  sciatic  participates 
in  the  innervation  of  the  adductor  magnus  muscle,  and  the  paralysis  of  the 
small  outer  rotators,  will  hardly  occasion  any  considerable  clinical  symptoms. 

The  disturbance  of  sensation  extends  also  into  the  area  of  the  cutaneus 
surae  lateralis  nerve  (external  saphenous  nerve). 

Especially  important  is  the  fact  that  in  high  seated  lesions  in  the  sciatic 
area,  partial  paralyses,  particularly  in  the  area  of  the  peroneus,  occur.  It  is 
almost  always  the  case  in  maternal  obstetric  paralysis,  that  any  existing 
more  extended  defects  soon  retrogress,  but  that  a  peroneal  paralysis,  which 
shows  a  decided  preference  for  the  area  of  the  ramus  profundus,  thus  sparing 
the  peronei  muscles,  remains.     A  great  many  attempts  have  been  made  to 


PROGNOSIS  AND  THERAPY  OF  SCIATIC  PALSY  163 

explain  this  peculiar  condition.  The  cause  for  the  last  mentioned  specific 
case  might  be  traced  to  a  certain  part  of  the  plexus  (which  was  mentioned 
in  the  introduction  as  the  lumbosacral  cord)  which  passes  into  the  sciatic  and 
which  perhaps  carries  fibres  for  the  ramus  profundus  peronei,  that  is  espe- 
cially exposed  to  mechanical  injuries  in  the  pelvis.  At  any  rate,  we  are  really 
confronted  with  a  greater  liability  to  lesion  in  the  peroneal  than  in  the  tibial 
nerve.  Whether  it  is  exposed  more  to  mechanical  injuries  in  other  places 
than  in  the  one  named,  whether  more  unfavorable  vascularisation  conditions 
obtain,  or  whether  still  other  factors  have  any  part  in  it,  is  unsettled.  An 
anatomic  fact,  which  may  some  time  acquire  clinical  interest,  is  the  following : 
even  though  the  fibres  destined  for  the  peroneus  and  tibialis  always  take 
their  course  as  far  as  the  upper  part  of  the  popliteal  space  in  closest  prox- 
imity, yet  they  are  frequently  to  be  found  far  up  from  the  point  of  their 
separation,  and  even  as  far  back  as  the  plexus  area,  as  separate,  or  easily 
separated  bundles. 

In  the  prognosis,  one  should  be  very  careful  not  to  interpret  too  opti- 
mistically those  sciatic  paralyses,  whose  point  of  lesion  must  be  looked  for 
in  the  deeper  part  of  the  pelvis.  Particularly  the  maternal  obstetric  paralysis 
leaves,  in  a  great  percentage  of  cases,  a  lasting  defect. 

In  separating  numerous  peripheral  paralyses  in  the  sciatic  area  with 
regard  to  differential  diagnosis  from  such  as  depend  upon  limited,  spinal, 
focal  affections,  peculiar  difficulties  will  be  encountered.  Our  knowledge 
concerning  the  way  in  which  the  nuclei  of  the  particular  muscles  are  grouped 
in  the  anterior  horns  of  the  spinal  cord,  is,  in  many  respects,  still  incomplete. 
At  all  events,  there  is  a  great  similarity  between  these  spinal  groups  and  the 
innervation  area  of  the  peripheral  nerves.  This  hint  should  be  sufficient 
to  warn  every  one  to  be  careful. 

In  supranuclear  paralysis  of  the  leg  produced  by  lesion  of  the  pyramidal 
tract,  the  extensors  of  the  foot  are  usually  injured  very  gravely.  This 
predilection  is  frequently  so  remarkable,  that  the  thought  of  a  peripheral 
complication  often  suggests  itself  at  once. 

Bilateral  peroneal  paralyses  are  usually  polyneuritic. 

Therapy. — We  call  particular  attention  to  prophylaxis  with  regard  to 
contractures,  which  is  so  very  necessary  in  peroneal  paralysis.  The  difficulty 
in  walking,  due  to  elevation  of  the  heel,  may  be  remedied  by  means  of  suitable 
apparatus,  which  partially  replaces  the  function  of  the  muscles  by  elastic 
straps.     For  tibialis  paralysis,  similar  apparatus  has  been  invented. 

Paralysis  of  the  gluteal  nerves  is  very  rare.  It  has  been  observed,  f.  i., 
after  fracture  of  the  os  sacrum  and  in  pelvic  tumors.  The  same  injury  may 
affect  the  posterior  cutaneous  nerve  of  the  thigh.  The  motor  paralysis 
affects  the  gluteal  muscles  and  the  tensor  fasciae  latse.  Extension  and  abduc- 
tion in  the  hip  joint  are  paralyzed;  and  the  inner  and  outer  rotation  greatly 
weakened.     When  walking,  the  lack  of  lateral  fixation  of  the  pelvis  against 


1 64  •  DISEASES  OF  THE  PERIPHERAL  NERVES 

the  thigh  is  especially  striking.  When  stepping  on  the  diseased  leg,  the 
pelvis  deviates  towards  the  sound  side;  the  movement  becomes  somewhat 
waddling. 

5.  Nerves  of  the  Pudendal  and  Coccygeal  Plexuses 

Radicular  paralyses  of  this  area  will  be  discussed  with  the  affections  of 
the  cauda  equina.  Next  to  nothing  is  known  concerning  affections  in  the 
area  of  the  real  plexuses  and  their  peripheral  branches. 

Supplement 

Occupation  Paralysis  and  Occupation  Neuritis 

As  we  have  seen,  typical  paralyses  of  single  peripheral  nerves  may  be 
caused  by  occupational  injuries.  Thus,  continual  pressure  produces  paraly- 
ses of  the  foot,  especially  of  the  peroneal  nerve,  in  persons  who  are  forced  to 
work  in  a  squatting  position,  paralyses  of  the  ulnar  in  persons  who  during 
their  work  continually  support  their  elbows  on  something,  while  pressure  and 
traction  produces  plexus  paralyses  in  those  who  carry  heavy  loads. 

One  group  of  cases,  however,  requires  a  separate  discussion,  namely,  that 
in  which  peculiar  clinical  pictures  are  occasioned  by  the  special  conditions 
of  occupational  aetiology,  and  cases  of  selective  injury  of  a  few  known 
muscles,  which  in  the  particular  occupation  are  usually  synchronously 
strained  and  compressed  or  otherwise  irritated  mechanically. 

The  most  important  form  practically,  affects  especially  workers  who 
must  handle  continuously  a  certain  tool,  the  handle  of  which  they  must  clasp 
tightly,  so  that  the  small  muscles  of  the  hand  are  both  strained  and  exposed 
to  pressure,  as  ironers,  locksmiths,  borers,  planers,  cutters  (who  must  use 
scissors)  and  workers  in  many  other  occupations.  Precisely  the  same  dis- 
turbance is  seen  in  persons  who  are  compelled  to  lean  with  one  hand  con- 
tinuously and  heavily  on  a  crutch.  In  all  these  cases  the  muscles  of  the 
ball  of  the  thumb  and  of  the  interosseus  primus  are  especially  endangered. 

Occupational  paresis  (which  is  similarly  localized)  of  milkers,  cigar- 
makers,  etc.,  may  mostly  be  traced  to  strain. 

We  shall  not  enumerate  the  rarer  analogous  occurrences,  except  the 
paralysis  of  a  few  muscles  of  the  left  thumb  of  drummers  (drummer-paral- 
ysis), in  diagnosing  which  particular  care  should  be  taken  not  to  confound  it 
with  a  surgical  affection  which  manifests  itself  under  the  same  circum- 
stances (rupture  of  the  tendon  of  the  extensor  longus  pollicis,  drummer's 
tendon). 

Besides  those  persons  who  by  reason  of  toxic  or  infectious  injuries  are 
predisposed  to  such  disease,  novices  who  do  their  work  with  an  unnecessary 
expenditure  of  force,  are  especially  liable  to  it. 


OCCUPATION  NEURITIS,  POLYNEURITIS  165 

The  clinical  picture  which  is  frequently  introduced  and  accompanied 
by  paresthesia  and  pains,  is  mainly  composed  of  weakness  of  the  affected 
muscles  or  atrophy,  which  sets  in  either  rapidly  or  stealthily. 

R.  D.  and  a  slight  decrease  of  sensibility  in  many  cases  indicate  that  the 
affection  is  neuritic.     Yet  myopathic  cases  possibly  occur  also. 

The  diagnosis  will  be  easy  if  proper  regard  be  given  to  the  aetiology. 
Even  disturbances  of  sensibility  and  pains,  when  present,  may  serve  to  dis- 
tinguish several  spinal  affections  having  externally  some  similarity.  Oc- 
cupation paresis  may  combine  with  occupation  neurosis. 

The  prognosis  of  the  pure  cases  is  usually  in  all  respects  favorable. 

Of  particular  importance  in  the  therapy  is  rest  for  the  affected  part,  and 
above  all,  of  course,  the  patient  must  temporarily  or  permanently  abandon 
the  injurious  occupation. 

Polyneuritis 

Polyneuritis  {Ley den,  about  1880)  is  a  more  or  less  widespread  primary 
degenerative  disease  of  the  peripheral  nervous  system,  distributed  nearly 
symmetrically  and  taking  a  regular  course,  and  developed  fundamentally 
from  a  general  injury,  resulting  from  toxic  matter  in  the  very  broadest  sense  of 
the  word,  with  which  the  bulk  of  the  bodily  fluids  have  become  contaminated. 

Hence  not  every  multiple  disease  of  peripheral  nerves  is  a  case  of  poly- 
neuritis. Such  diseases  in  which  a  multiple  paralysis  of  the  cranial  nerves 
or  of  the  roots  is  occasioned  by  gummatous  infiltration  or  sarcomatous 
tumors  in  the  area  of  the  meninges,  of  the  base  of  the  skull  or  of  the  spinal 
cord,  are  on  the  best  of  grounds  not  considered  as  polyneuritis.  Nor  would 
multiple  paralyses  resulting  from  tumors  or  gummatous  processes  on  the 
peripheral  nerves  themselves  come  under  such  classification.  Other  cases 
are  much  more  akin  to  polyneuritis.  We  have  observed  before,  that  upon  a  sub- 
stratum of  general  injuries,  which  are  usually  at  the  bottom  of  polyneuritis 
there  frequently  develop  isolated  paralyses  of  peripheral  nerves.  Paralyses 
of  several  single  nerves,  the  irregular  choice  of  which  is  presumably  due  to 
specific  local  causes  also  occur  rather  often  under  such  greatly  predisposing 
conditions.     These  cases  must  likewise  be  distinguished  from  polyneuritis. 

On  the  other  hand,  however,  local  factors,  such  as  heavy  functional  de- 
mands on  certain  parts,  may  in  some  respects  modify  the  distribution  even  in 
polyneuritis  proper.  Nevertheless  it  will  be  necessary  and  almost  always 
possible  to  separate  distinctly,  both  theoretically  and  practically,  the  poly- 
neuritis, the  symmetric  polyneuritis,  from  other  multiple  nerve  paralyses 
(multiple  mononeuritis  or  polyneuritis  disseminata  and  multiple  tumor 
formations) . 

Etiology. — The  majority  of  cases  of  polyneuritis  in  Germany  are  occa- 
sioned by  chronic  alcoholism.     Next  to  this  the  form  known  as  lead  paralysis 


166  DISEASES  OF  THE  PERIPHERAL  NERVES 

is  probably  of  greatest  practical  importance.  In  the  majority  of  cases  chronic 
poisonings  sustained  during  work  with  lead  occasion  lead  paralysis,  but  such 
diseases  have  been  seen,  also,  after  all  kinds  of  more  casual  poisonings. 
Among  the  classes  of  workmen  who  are  thus  endangered  are  those  who  are 
engaged  in  the  metallurgical  extraction  of  lead;  those  who  cast  the  metal 
into  shot,  pipes,  type  (type  founders),  or  use  it  for  chemical  preparations, 
such  as  colors;  also  painters,  varnishers  and  dyers  who  use  lead  colors,  potters 
who  apply  glaze  containing  lead,  compositors  who  set  lead  type;  gold  leaf 
workers,  filecutters  and  engravers  who  use  lead  plates  as  supports;  workers 
who  must  handle  the  lead  plates  used  in  storage  batteries,  plumbers  and 
others  who  solder  pipes  with  lead  alloys  and  tinners  who  use  lead  for  the 
same  purpose. 

Arsenical  paralysis,  after  an  improper  medical  use  of  arsenic,  is  the  next 
frequent  toxic  polyneuritis.  Polyneuritis  from  bisulphide  of  carbon  poison- 
ing is  seen  in  some  rare  cases  in  workers  employed  in  the  vulcanizing  rooms 
of  rubber  factories.  Toxic  polyneuritis  after  poisoning  from  carbonic  oxide, 
mercury  and  some  other  poisons  is  a  great  rarity. 

Polyneuritis  accompanying  and  following  infectious  diseases  forms  a 
second  important  group.  Of  greatest  practical  interest  is  undoubtedly  the 
so-called  post-diphtheritic  paralysis,  which  most  frequently  develops  about 
the  third  week  of  convalescence.1  In  connection  with  typhoid,  influenza, 
malaria  and  other  acute  infections  polyneuritis  is  much  rarer.  In  these  cases 
the  nerve  affection  also  usually  sets  in  during  the  convalescent  stage  of  the 
primary  disease.  In  syphilis,  polyneuritis  manifests  itself  in  the  secondary 
stage,  generally  not  later  than  half  a  year  after  the  infection.  It  also  occurs 
occasionally  in  the  course  of  gonorrhea  and  in  advanced  pulmonary  tuber- 
culosis. We  shall  not  enumerate  here  the  rarer  infectious  causes.  In  a 
supplement  to  this  chapter  we  shall  briefly  consider  the  nerve  affection  in 
leprosy. 

Besides  the  main  astiological  groups  of  toxic  polyneuritis  and  those 
appearing  in  connection  with  infectious  diseases,  the  so-called  cachectic- 
dyscrasic  forms  occupy  practically  only  a  subordinate  place.  Those  cases 
occasionally  seen  in  various  marasmic  conditions  and  in  metabolic  diseases, 
as  diabetes,  and  polyneuritis  manifesting  itself  during  pregnancy  or  when 
lying-in,  unless  it  can  be  referred  to  an  accompanying  infectious  disease 
(puerperal  fever),  are  classed  among  the  above  mentioned  forms. 

In  quite  a  number  of  cases  polyneuritis  has  to  be  designated  as  idiopathic 
for  want  of  detectable  aetiological  factors.  It  should  not  be  forgotten  that 
even  in  the  toxic,  infectious  and  cachectic-dyscrasic  forms  the  immediate 
cause  of  the  disease  is  entirely  obscure,  for  it  is  only  in  a  small  percentage  of 

1  The  prejudice  of  the  laity  that  diphtheritic  paralysis  is  a  result  of  the  serum  treatment  has 
no  foundation  whatsoever.  On  the  contrary,  it  seems  as  if  serum  treatment  instituted  very  early 
lessens  the  danger  of  an  occurrence  of  this  disease.     (Heubner.) 


THE  CLINICAL  PICTURE  OF  POLYNEURITIS  167 

those  affected  with  such  injuries  that  a  polyneuritis  really  develops.  It  is 
not.  infrequent,  therefore,  that  even  such  a  predisposing  factor  can  not  be 
found  with  certainty.  Some  of  such  cases  give  the  impression  of  an  inde- 
pendent infectious  disease. 

A  particular,  probably  infectious,  form  of  disease,  endemic  in  Eastern 
Asia,  Brazil  and  Africa,  which  sometimes  spreads  even  into  Germany,  is 
called  beri-beri. 

It  remains  only  to  be  added,  that  in  the  aetiology  of  polyneuritis  several 
of  the  factors  mentioned  may  concur  and  that  a  cold  may  in  many  cases  be 
an  exciting  factor. 

The  clinical  picture  of  polyneuritis  is  composed  of  numerous  peripheral 
nerve  paralyses  and  nerve  pareses  distributed  more  or  less  strictly  sym- 
metrically over  both  sides  of  the  body. 

In  the  following  more  minute  description  we  begin  with  a  fundamental 
type,  such  as  for  instance  in  alcoholic  polyneuritis  and  in  the  majority  of 
the  other  forms  constitutes  the  rule,  and  then  continue  by  discussing  the 
clinical  peculiarities  of  some  special  cases. 

In  the  great  majority  of  cases  the  nerves  of  the  leg,  and  among  these,  the 
peroneal  nerves,  are  affected  first  and  most  severely.  Frequently  only  one 
leg  is  affected  at  first  but  very  soon  the  other  succumbs.  Next  to  the 
peroneal  the  tibial  nerves  are  affected,  as  a  rule.  Ascending,  the  disease 
then  seizes  upon  the  nerves  of  the  thigh  and  the  pelvis,  and  usually  together 
with  them,  or  even  before  them,  also  a  part  of  the  nerves  of  the  upper  extrem- 
ities, in  most  cases  first  the  musculo-spiral,  then  the  median  and  ulnar  nerves. 
In  the  upper  extremities  too,  a  partiality  for  the  distal  parts  manifests  itself. 
Less  often  are  the  shoulder  girdle  and  the  musculature  of  the  upper  arms 
affected.  The  supinator  longus  muscles  of  the  radial  nerve's  innervation 
area  are  also  not  infrequently  spared.  Cases  in  which  the  paralysis  of  the 
arms  bears  the  character  of  a  plexus  paralysis  are  very  uncommon. 

As  a  rule,  the  paralysis  does  not  develop  with  equal  severity  in  the 
various  nerve  areas.  A  complete  paralysis  occurs  most  frequently  in  the 
musculature  of  the  leg.  In  other  nerve  areas,  it  is  often  only  a  case  of  a 
more  or  less  serious  paresis.  In  grave  cases,  almost  or  quite  complete, 
paralysis  of  all  four  extremities  may  indeed  occur,  and  in  such  cases,  the 
muscular  apparatus  of  the  trunk,  the  abdominal  musculature,  and  the 
musculature  of  the  back  and  neck  will  not  often  be  spared. 

Usually  it  soon  becomes  possible  to  recognize  the  atrophy  of  the  affected 
muscle-groups.  Electric  stimulation  indicates  what  changes  are  to  be 
expected ;  generally  it  will  be  R.  D .  Almost  always  the  reflexes  are  very  much 
weakened  or  destroyed.  Destruction  of  the  tendon  and  periosteal  reflexes 
may  be  one  of  the  earliest  symptoms.  Only  rarely  can  a  transient  initial 
exaggeration  of  the  reflexes  be  noted.  Even  in  those  light  cases  in  which  the 
tibial  and  crural  nerves  are  comparatively  slightly  affected,  it  is  unusual  for 


1 68  DISEASES  OF  THE  PERIPHERAL  NERVES 

the  knee  jerk  and  Achilles  tendon  reflexes  to  be  preserved  all  through  the 
illness.  More  often  an  exaggeration  of  the  cutaneous  reflexes  (plantar 
reflexes),  particularly  in  hyperalgesic  cases,  sets  in. 

By  the  paralyses  the  members  are  placed  in  anomalous  positions.  The 
feet  are  in  the  pes  equinus  position;  the  hands  usually  show  wrist-drop,  or  in 
some  cases,  the  ape  hand  or  talon  position,  or  a  mixed  form.  In  protracted 
duration  of  the  affection,  the  anomalous  positions  are  fixed  by  contractures, 
unless  treatment  prevents  it. 

"When  the  patient  is  still  able  to  walk,  or  as  soon  as  he  has  regained  that 
ability,  a  pronounced  paretic  gait,  steppage  gait  or  "waddling  gait,"  accord- 
ing to  the  extent  and  distribution  of  the  affection,  will  be  observed.  Among 
the  earliest  symptoms  of  many  cases  of  disorders  occurring  in  the  sensory 
area,  are  paraesthesia,  formication,  and  a  sense  of  numbness  particularly  in 
the  distal  parts  of  the  extremities,  beginning  in  the  feet.  Frequently,  but 
by  no  means  always,  very  irritating,  drawing,  burning  pains  manifest  them- 
selves in  the  parts  affected,  while  the  nerve  trunks  and  the  musculature 
are  sensitive  to  pressure.  When  the  pains  are  severe,  and  the  paralysis  is 
not  total,  the  patient  is  wont  to  resist  passive  movements,  which  sometimes 
misleads  the  inexperienced  into  thinking  that  he  is  dealing  with  spastic 
conditions. 

Objective  disorders  of  sensation  are  of  far  less  importance  than  motor 
disorders,  and  even  in  grave  cases  sometimes  confine  themselves  to  a  few 
nerve  areas  of  the  distal  parts  of  the  extremity.  However,  it  is  very7  unusual 
for  them  to  be  lacking  entirely.  Especially  in  alcoholic  and  arsenical 
paralyses  a  rather  extensive  hyperalgesia  of  the  skin  sometimes  occurs, 
besides  the  decrease  of  the  other  sense  qualities  commonly  restricted  within 
narrower  bounds. 

Disorders  in  the  secretion  of  sweat,  hyperhidrosis  or  anhidrosis,  reddening 
of  the  skin,  and  a  glossy  skin,  are  among  the  trophic  and  vasomotor-secretory 
disorders  occurring  more  frequently.  Considerable  desquamation  and  now 
and  then  edema  are  also  seen. 

The  participation  of  the  phrenic  and  of  the  cranial  nerves  is  one  of  the 
rarer  symptoms  of  polyneuritis.  Facial,  eye  muscle  and  vagus  paralyses  in 
particular,  less  frequently  optic,  auditor}7  and  other  forms  of  neuritis,  are  now 
and  then  observed. 

Very  unusual  are  disorders  of  innervation  affecting  the  bladder  and 
rectum. 

Xot  infrequently  persons  affected  with  polyneuritis  show  symptoms  of  a 
psychosis.  A  certain  symptom-complex  of  psychic  disorders  has  in  fact  been 
designated  as  polyneuritis  psychosis  (Korsakow).  It  is  undoubtedly  true 
that  it  is  found  very  frequently  in  persons  affected  with  polyneuritis.  It  is 
particularly  characterized  by  a  serious  disorder  of  the  ability  to  receive  and 
retain  new  impressions.     Patients  who  often  can  still  recollect  events  of  the 


PARTICULAR  CLINICAL  FORMS  OF  POLYNEURITIS       169 

more  remote  past,  are  entirely  incapable  of  giving  a  correct  account  of  the 
events  of  the  immediate  past,  or  even  the  last  few  hours.  The  resulting  void 
in  the  memory  is  then  filled  with  "fairytales"  (confabulation).  So,  e.  g.,  a 
patient  who  for  weeks  had  been  confined  to  his  bed  with  paralysis,  told  the 
doctor  who  was  making  his  morning  round,  that  he  had  already  taken  a  walk 
in  the  morning,  and  had  then  eaten  his  dinner  with  much  relish. 

Particular  Clinical  Forms 

1.  Pseudotabes  Peripherica. — This  form,  found  especially  in  alcoholics, 
but  also  after  diphtheria,  in  those  affected  with  diabetes  and  occasionally 
from  other  causes,  is  characterized  by  the  fact  that  its  symptoms  of  lost 
function  manifest  themselves  preponderantly  in  the  sensory  area.  Among 
the  earliest  subjective  complaints  of  the  patient  is  a  motor  ataxia — which  has 
a  strong  resemblance  to  the  tabetic  one — of  the  lower,  later  perhaps  also 
of  the  upper,  extremities.  This  ataxia,  like  all  others,  depends  in  particular 
on  extensive  disorders  of  the  deeper  sensibility.  In  such  cases,  anaesthesias 
of  the  skin  occur  more  pronouncedly  than  in  other  cases,  and  are  localized 
corresponding  to  the  ramifications  of  the  cutaneous  nerves.  The  Romberg 
phenomenon  is  present;  the  reflexes  are  destroyed.  Symptoms  of  motor 
weakness,  on  the  other  hand,  play  only  a  secondary  role,  but  at  least  in  a  few 
nerve  areas  (e.  g.,  the  peroneal  area)  they  will  hardly  ever  be  entirely  absent. 

2.  Lead  paralysis,  in  the  great  majority  of  cases,  and  especially  as  a 
rule  in  adults,  is  confined  to  the  upper  extremities.  It  first  affects  the  musculo- 
spiral  nerve,  usually  starting  with  the  right,  and  going  over  to  the  left,  in  a  very 
peculiar  selective  fashion,  first  affecting  the  extensors  of  the  fingers  and  those 
of  the  wrist.  The  disease  may  be  restricted  to  this  narrow  area.  If  it 
progresses,  it  next  seizes  upon  the  long  abductors  of  the  thumb  and  the  small 
muscles  of  the  hand  supplied  by  the  ulnar  and  median  nerves.  A  further 
extension  to  brachio-radial,  supinator,  and  other  arm  muscles,  is  quite  un- 
common, while  a  generalization  of  the  paralysis  is  a  true  rarity.  A  predi- 
lection of  the  paralysis  for  the  lower  extremity,  in  particular,  for  parts  of  the 
peroneal  area,  is  most  likely  to  be  found  in  children.  The  sensibility  almost 
always  remains  unimpaired. 

This  most  peculiar  picture  undoubtedly  indicates  a  spinal  rather  than  a 
neuritic  disease  (Erb,  E.  Remak).  But  the  numerous  existing  anatomical 
reports  do  not  permit  any  doubt  as  to  its  neuritic  character.  The  peculiar 
manner  of  localization  is  still  a  matter  of  controversy. 

3.  Postdiphtheritic  paralysis  is  also  distinguished  by  a  peculiar 
localization.  Almost  without  exception,  a  motor  paralysis,  and  sometimes 
an  anaesthesia  of  the  soft  palate  (velum  palatinum)  is  the  first  and  often  the 
only  symptom.  The  speech  of  the  patients  is  openly  nasal;  liquids  that  they 
are  drinking  partly  flow  back  through  the  nose.     Frequently  a  paralysis  in 


170  DISEASES  OF  THE  PERIPHERAL  NERVES 

accommodation  accompanies  the  soft  palate  paralysis,  to  which  it  is  interest- 
ing to  note,  the  hyperopics  especially,  who  read  much  during  convalescence, 
are  said  to  be  predisposed. 

In  some  cases,  still  other  eye  muscles,  especially  the  external  recti,  the 
muscles  of  the  oesophagus,  the  vagus  branches  in  the  larynx,  and  other  areas 
of  cranial  nerves  become  affected.  More  frequent  is  a  participation  of  the 
extremities.  Sometimes  its  only  indication  is  the  destruction  of  the  tendon 
reflexes  of  the  legs.  In  other  cases,  a  typical,  polyneuritic  paralysis  of  the 
upper  and  the  lower  extremities  sets  in,  and  even  further  generalizations 
occur.  Instead  of  that,  however,  the  picture  of  pseudotabes  in  some  cases 
combines  with  the  palatal  and  accommodation  paralysis. 

One  is  inclined  to  regard  the  paralysis  of  the  palate  after  diphtheria  not 
as  a  polyneuritic  symptom,  but  as  a  kind  of  ascending  neuritis,  especially  as 
after  diphtheria  of  the  umbilicus,  a  paralysis  of  the  abdominal  muscles  has 
been  observed.  Yet  on  the  other  hand,  paralysis  of  the  soft  palate  and  pa- 
ralysis in  accommodation  have  been  seen  to  set  in  after  diphtheritic  infec- 
tions in  the  arm. 

The  diagnosis  of  polyneuritis  is  in  most  cases  easy.  Leaving  the 
typical  postdiphtheritic  and  lead  paralysis,  which,  especially  with  a  knowl- 
edge of  their  aetiology,  can  hardly  be  mistaken,  out  of  consideration,  the  follow- 
ing signs  of  the  clinical  picture  will  serve  especially  to  distinguish  polyneuritis 
from  other  extensive  paralyses  and  paralytic  conditions:  its  symmetrical 
distribution;  its  incipiently  progressive,  as  a  rule  ascending,  course  which 
later  has  a  tendency  to  recovery;  the  nature  of  the  symptoms  of  lost  function; 
the  paralyses  connected  with  R.  D.  besides  the  disorders  of  sensation  which 
only  rarely  will  be  absent;  and,  lastly,  the  almost  always  obvious  grouping 
of  disorders  corresponding  to  the  innervation  area  of  peripheral  nerves.  Nor 
is  the  psychosis  of  Korsakow  found  in  those  affections  which  are  of  any 
differential  diagnostic  importance. 

We  shall  now  mention  the  most  important  special  differentiating  signs 
of  those  diseases,  which  at  one  time  or  another,  are  most  likely  to  be  mis- 
taken for  polyneuritis. 

In  acute  poliomyelitic  paralysis  the  progressive  course  is  lacking.  It 
sets  in  with  all  its  gravity,  and  then  retrogresses.  Only  in  very  exceptional 
cases  is  its  distribution  symmetrical.  Disorders  of  sensation  are  almost 
always  absent.  Spinal  progressive  muscular  atrophies  are  characterized  by 
their  more  creeping  progress,  by  the  lateness  at  which  functional  disorders 
are  observed,  by  fibrillary  twitchings  which  are  very  unusual  in  peripheral 
diseases,  and  also  by  the  absence  of  disorders  of  sensation.  Concerning 
the  differentiation  (which  sometimes  becomes  difficult)  from  the  so-called 
neuritic  progressive  muscular  dystrophy,  a  disease  which  likewise  sets  in 
very  slowly  and  chronically,  we  refer  to  the  chapter  in  this  work  dealing 
with  it. 


DIFFERENTIAL  DIAGNOSIS  OF  POLYNEURITIS  171 

Genuine  tabes  is  distinguished  from  the  ataxic  form  of  polyneuritis  by  a 
number  of  specific  traits,  foremost  among  which  is  reflex  pupillary  immobil- 
ity; but  also  by  other  signs,  some  of  which  set  in  very  early,  such  as  gastric 
crises,  primary  atrophy  of  the  optic  nerve,  bladder  disorders,  which  are  very 
rare  in  polyneuritis,  and,  of  course,  by  the  segmental  arrangement  of  the 
sensory  losses.  On  the  other  hand,  the  paresis,  which  will  hardly  ever  be 
altogether  lacking  in  typical  tabes  is  absent  in  pseudotabes.  In  tabes  there 
often  exists  an  analgesia  of  the  nerve  trunks  to  pressure;  in  pseudotabes, 
there  is  an  abnormal  sensitiveness  to  pressure. 

Myopathic  diseases,  which  may  lead  to  extensive,  likewise  symmetrically 
distributed,  disorders  of  motility  (progressive  dystrophies,  polymyositis, 
osteomalacial  paralysis,  myasthenia),  lack  the  symptoms  which  indicate 
directly  a  disease  of  the  nerves,  namely  R.  D.,  early  decrease  or  destruction 
of  reflexes,  and  disorders  of  sensation.  We  need  not  mention  here  how  each 
individually  is  often  revealed  at  a  glance  by  certain  positive  signs  to  those 
skilled  in  them.  We  refer  only  to  the  peculiar  type  of  localization  in  the 
various  forms  of  dystrophy  and  to  the  inflammatory  swelling  of  the  muscles, 
and  in  polymyositis,  frequently  of  the  skin  also. 

Chiefly,  of  course,  in  the  interest  of  successful  treatment,  a  complete 
diagnosis  of  polyneuritis  naturally  requires  that  the  aetiology,  founded  upon 
objective  criterions,  be  determined  as  accurately  as  possible.  Hence  it  will 
be  necessary  to  look  for  objective  signs  of  chronic  alcoholism  (psychic 
changes,  tremor);  of  lead  poisoning  (the  blue  line  on  the  gums,  basophilic 
granulation  of  the  erythrocytes,  lead  in  the  urine,  which  in  some  cases  will 
be  revealed  only  after  administering  iodine);  of  an  abuse  of  arsenic  (cuta- 
neous alterations,  melanosis);  of  CS2  poisoning  (psychic  alterations); 
and  for  signs  of  a  former  or  existing  infectious  disease  or  metabolic 
affection. 

In  the  treatment  of  a  diphtheritic  paralysis,  the  frequently  occurring 
myocarditic  complication  must  be  taken  into  consideration.  In  the  disease 
known  as  beri-beri,  a  myocarditis,  taking  the  same  course  as  the  symptoms 
of  circulatory  weakness,  particularly  edemas,  is  really  part  of  the  clinical 
picture. 

Course  and  Termination.  Prognosis. — Polyneuritis  may  set  in 
acutely,  even  with  fever,  or  may  develop  slowly.  In  some  rare  cases,  the 
disease  progresses  rapidly  from  day  to  day,  and  soon  reaches  its  highest 
point  of  development.  More  frequently,  however,  the  symptoms  require  a 
number  of  weeks,  or  even  months  for  their  full  development.  The  more 
chronic  development  progresses  steadily,  or,  sometimes,  by  fits  and  starts. 
Carelessness  of  the  patient,  such  as  excessive  indulgence  in  alcohol,  may  at 
times  lead  to  an  exacerbation  of  the  symptoms. 

It  has  already  been  pointed  out  in  the  discussion  of  symptomatology, 
that  different  cases  attain  to  varying  degrees  of  extension.     Some,  in  fact, 


172  DISEASES  OF  THE  PERIPHERAL  NERVES 

remain  rudimentary.     In  the  form  described  as  the  fundamental  type,  the 
affection  sometimes  does  not  spread  beyond  the  lower  extremities. 

The  prognosis  in  general  is  usually  exceedingly  favorable. 

A  fatal  issue,  caused  by  the  affection  alone,  is  a  rare  exception.  Some 
cases  taking  a  highly  acute  and  rapidly  ascending  course,  do,  indeed, 
result  in  death  in  a  short  time.  Paralyses  of  bulbar  nerve  areas,  and  of 
respiration,  which  latter  are  often  of  such  nature  as  to  be  difficult  of  explana- 
tion, frequently  cause  death.  Even  in  cases  taking  a  less  violent  course, 
bilateral  phrenic  and  vagus  paralyses  may  threaten  life  by  their  various 
attendant  dangers.  It  is  hardly  necessary  to  mention  that  for  persons  whose 
general  condition  is  wretched,  the  length  of  the  disease  may  become  serious, 
or  that  the  gravity  of  the  primary  disease  may  endanger  life. 

The  great  majority  of  cases,  as  we  have  already  said,  recover.  Some- 
times a  complete  restoration  takes  place  after  a  few  weeks.  Even  in  wide- 
spread cases  this  occurs,  if  the  paralyses  and  the  alterations  of  electric 
irritability  do  not  attain  a  very  high  degree.  But  in  most  cases,  nevertheless, 
it  takes  many  months  or  even  longer  in  grave  cases,  to  recover.  Lasting 
defects  may  usually  be  avoided. 

Naturally  much  depends  on  the  possibility  of  completely  eliminating 
the  causal  injuries.  Alcoholics  who  do  not  renounce  alcohol,  those  affected 
with  lead  poisoning  who  continue  to  receive  the  poison  into  their  system, 
cannot  hope  to  recover. 

Only  a  few  words  concerning  the  pathological  anatomy  are  necessary 
to  supplement  the  discussion  in  the  general  part.  It  is  a  matter  of  a  primary 
degenerative  process,  a  parenchymatous  neuritis.  The  peripheral  ramifica- 
tions of  the  nerves  are  more  likely  to  be  seriously  affected  than  the  large 
trunks  and  roots.  It  seems  that  the  fibre  disease,  which  is  typical  of  poly- 
neuritis, is  the  degenerative  form  beginning  in  the  periaxial  medullary 
sheaths.  Slight  changes  of  a  like  nature  are  frequently  found  in  nerve  areas, 
which  have  not  given  evidence  of  any  symptoms  of  lost  function  (latent 
neuritis).  The  unequal,  intermittent  extension  of  this  destructive  process 
makes  it  possible  that  Wallerian  degeneration  sets  in  in  a  fibre  segment  which 
at  first  remained  normal,  when  the  change  in  the  neighboring  proximal 
parts  has  progressed  to  the  destruction  of  the  axon.  One  is  inclined  to 
explain  in  this  manner  the  discovery  of  fibres  which  show  the  Wallerian 
degeneration  type,  in  seriously  diseased  nerves.  In  cases  of  long  standing 
numerous  fibres  disappear  wholly  (Figs.  38  and  39). 

The  changes  in  the  connective  tissue  of  the  nerve,  as  a  rule,  appear  to 
be  secondary.  Pure  inflammatory  focal  affections  of  the  nerve  branches 
and  trunks  are  found  now  and  then,  but  they  are  always  quite  circumscribed 
in  extent  and  are  an  inconstant  accompanying  symptom,  showing  nothing 
typical. 

In  the  spinal  cord  and  spinal  ganglia  are  often  found  those  slight  changes, 


THERAPY  OF  POLYNEURITIS 


173 


Fig.  38. — Fresh  polyneuritic  degenera- 
tion. (After  Balz  and  Miura.)  Teased 
preparation  of  the  peroneal  nerve  in  a  case 
of  beri-beri.  Osmium  staining.  In  some 
fibres  pronounced  periaxial  medullary  dis- 
integration; in  others  apparently  Wallerian 
degeneration. 


closely  related  to  peripheral  nerve  diseases,  which  have  been  discussed  in 
the  general  part.  In  passing  we  may  remark  that  besides  these,  complicating 
focal  affections  of  the  brain  and  spinal  cord  may  sometimes  occur  in  some 
forms  of  polyneuritis,  as  inflammatory  and  other  cerebral  focal  affections  in 
diphtheria,  and  the  so-called  encephalo- 
pathia  saturnina  in  lead  poisoning. 

The  therapy  of  polyneuritis  requires 
but  a  brief  discussion,  since  the  impor- 
tant general  rules  which  apply  to  poly- 
neuritis as  well,  have  been  dealt  with  at 
length  in  the  discussion  of  the  paralyses 
of  the  single  nerves.  Because  of  this 
we  may  pass  over  the  requirements  of 
absolute  rest  in  new  cases,  of  a  suitable 
diet,  of  proper  position,  in  connection 
with  which  especially  the  prophylaxis  of 
passive  contractures  should  be  con- 
sidered and  of  other  requirements. 

In  order  to  treat  the  causal  conditions  properly,  we  should  insist  on  total 
abstinence  in  alcoholics,  eliminate  the  poison  in  those  affected  with  lead 
poisoning  by  administering  iodide  of  potassium  (2  to  3  times  daily  0.5  (5  grs.) 

in  solution) ,  and  treat  malaria  with 
quinine,  diabetes  with  a  proper 
diet  and  syphilis  energetically  with 
mercury.  Any  previous  inunctions 
should  not  be  regarded  as  inter- 
fering with  this,  unless,  indeed, 
symptoms  of  mercurial  poisoning 
should  exist.  Some  cases  which  in 
spite  of  the  treatment  continue  to 
progress  in  the  beginning,  will  often 
recover  completely  in  a  remarkably 
short  time  if  the  treatment  is  con- 
tinued longer. 

No  definite  rule  can  at  present 
be  established  concerning  the  diffi- 
cult question  of  interrupting  preg- 
nancy in  pregnancy  polyneuritis 
(cf.  von  Hosslin,  Arch.  f.  Psychia- 
trie,  vol.  xl,  and  Munchner  med.  Wochenschr.,  1905,  page  636). 

For  the  rest,  the  use  of  moist,  warm  packings  of  the  whole  body,  or  of  the 
diseased  parts  is  to  be  recommended  in  the  early  stage.  They  may  be 
applied  daily  from  1  to  2  hours  and  will  almost  invariably  prove  beneficial, 


Fig.  39. — Polyneuritis  alcoholica  in  a  later 
stage.  (After  Jacob.)  Cross-section  of  the  n. 
ischiadicus.  In  the  proliferated  connective  tissue 
only  a  few  isolated  (black  colored)  fibers  are 
preserved. 


174  DISEASES  OF  THE  PERIPHERAL  NERVES 

particularly  in  painful  cases.  Suitable  for  every  stage — in  the  earliest  for 
those  patients,  at  least,  who  are  not  too  sensitive — are  thermal  baths  of 
960  to  980  F.,  continued  on  the  average  for  half  an  hour,  taken  3  to  6  times  a 
week.  Sometimes  medicinal  preparations  (pine  needle  extract,  salt,  carbonic 
acid,  etc.) ,  may  be  added.  Frequent  diaphoretic  procedures  such  as  hot  air 
baths  in  the  bed,  are  also  urgently  recommended  for  both  the  early  and  later 
stages  of  the  disease  (Oppenheim) . 

The  tonic  pills  mentioned  before  should  be  used  rather  than  other  med- 
icinal remedies,  to  aid  the  general  treatment. 

For  the  pains,  antineuralgic  agents  or,  if  necessary,  also  the  stabile  anodic 
galvanization  are  used  besides  warm  local  applications. 

When  the  affection  does  not  progress  any  further,  and  especially  during 
convalescence,  the  other  electro-therapeutic  means,  massage,  and  passive 
and  active  gymnastics  should  be  used  according  to  the  rules  mentioned  above. 
Careful  passive  movements  may  sometimes  prove  advantageous  even  in  the 
earlier  stage. 

As  after  cures,  thermal  bathing  resorts  furnished  with  good  medico- 
mechanical  institutions  are  particularly  to  be  recommended. 

Careful  treatment  will  obviate  the  necessity  of  having  to  remove  any 
residual  defects  by  means  of  orthopedic  surgery. 

Naturally  any  grave  special  occurrences  during  the  course  of  the  disease 
will  require  special  treatment.  In  deglutition  paralyses  it  is  better  to  have 
recourse  to  artificial  feeding  than  to  risk  an  aspiration  pneumonia.  In  grave 
acute  disorders  of  respiration,  the  life  of  the  patient  may  be  saved  by  artificial 
respiration  (Ebstein). 

Supplement 

A.  Landry's  Paralysis,  Paralysis  Ascendens  Acuta 

As  Landry's  paralysis  (Landry,  1859)  all  those  cases  are  commonly 
designated,  in  which  a  severe  and  flaccid  paralysis  begins  acutely  in  the 
feet,  thence  progresses  rapidly  and  steadily  to  all  remaining  muscles  of  the 
legs,  trunk,  arms,  neck  and  the  bulbar  nerve  areas.  In  sporadic  cases  the 
onset  of  the  disease  is  marked  by  a  febrile  attack.  The  reflexes  are  destroyed 
and  usually  there  are  slight  disorders  of  sensibility.  As  a  rule,  no  real  pains 
are  experienced.  Disorders  of  bladder  or  rectum  are  found  only  exception- 
ally. Death  generally  results  in  the  first  few  days  or  the  second  week  (rarely 
later)  from  suffocation  or  respiratory  failure.     Some  cases  recover  slowly. 

A  descending  course  of  the  disease  has  also  been  described. 

It  is  undoubtedly  true  that  a  polyneuritis  acutissima  may  occasion  this 
clinical  picture.  But  it  would  be  wrong,  simply  to  identify  Landry's  paralysis 
with  polyneuritis.  Some  postmortem  findings  seem  to  indicate  that  it  may 
also  be  caused  by  an  ascending  myelitis.     Besides  there  are  some  cases  in 


LEPROSY  OF  THE  XERVOCS  SYSTEM  175 

which  a  remarkable  condition  of  electric  irritability  exists  which  is  incom- 
patible with  either  polyneuritis  or  myelitis.  Autopsies  very  rarely  afford  a 
satisfactory  explanation  of  the  clinical  picture. 

At  any  rate  it  is  a  matter  of  a  toxic  or  infectious  disease.  It  may 
appear  in  connection  with  certain  infectious  diseases  and  toxic  processes. 
Swelling  of  the  spleen  and  albuminuria  may  follow  in  its  wake.  In  not  a 
few  cases  micro-organisms  of  various  kinds  have  been  found  in  the  tissues 
of  the  body,  particularly  in  the  central  organs. 

It  is  at  present  impossible  to  distinguish  clearly  from  each  other  and  to 
classify  the  cases  of  Landry's  paralysis. 

B.  Leprosy  of  the  Nervous  System 

Leprosy  of  the  nervous  system  is  now  and  then  very  erroneously  simply 
designated  as  polyneuritis  leprosa. 

Grave  disorders  of  cutaneous  sensibility,  in  its  earlier  stages,  as  a  rule, 
extending  irregularly  in  spots,  or  appearing  in  segmental  radicular  zones  and 
afterwards  covering  extensive  areas,  even  the  whole  body,  sometimes 
affecting  all  qualities,  sometimes  only  one  or  more,  stand  in  the  foreground 
of  the  clinical  picture  (lepra  ana?sthetica\  The  sensibility  of  the  deeper 
parts  usually  remains  unimpaired,  for  which  reason  ataxia  hardly  ever 
occurs.  The  anaesthesias  combine  with  muscle  atrophies,  usually  only  in 
limited  areas,  among  which  there  is  a  predilection  for  the  small  muscles  of 
the  hand,  and  with  grave  ''trophic"  disorders,  which  partly  depend  un- 
doubtedly upon  the  nerve  disease  and  partly  constitute  symptoms  of  local 
leprous  changes  leading  even  to  loss  of  entire  ringer  joints  (lepra  mutilans). 
The  state  of  the  reflexes  is  always  irregular.  Typical  paralyses  of  peripheral 
nerves  appear  only  in  exceptional  cases. 

It  is  obvious  that  this  clinical  picture  may  be  mistaken  for  syringomyelia 
more  easily  than  for  polyneuritis,  despite  the  fact  that  both  halves  of  the 
body  may  be  affected  almost  symmetrically.  It  may  be  very  difficult  to 
distinguish  it  from  syringomyelia.  But  usually  the  characteristic  spotted 
and  nodular  leprous  changes  of  the  skin  will  be  found  in  leprosy  of  the 
nervous  system,  while  in  syringomyelia  a  few  symptoms,  like  the  spastic 
palsy,  will  manifest  themselves,  which  as  a  rule  are  not  found  in  leprosy. 

The  pathological-anatomic  explanation  of  the  picture  of  "lepra  ner- 
vorum" is  not  at  all  conclusive  as  yet.  It  is  certain  that  alterations  of 
minute  nerve  branches  of  the  skin  together  with  leprous  cutaneous  diseases 
play  a  role  in  it.  But  besides  these,  there  are  also,  perhaps  ascending, 
bacillary  invasions  of  the  larger  trunks,  the  spinal  ganglia  and  the  spinal 
cord,  with  or  without  consecutive,  especially  interstitial,  changes  (neuritis 
interstitialis) .  Occasionally  lepra  nodules  on  the  larger  nerve  trunks  destroy 
them.     Just  how  often  primary  polyneuritic  degeneration  of  the  nerves 


176  DISEASES  OF  THE  PERIPHERAL  NERVES 

occurs  in  such  cases  as  a  result  of  a  general  toxaemic  disposition,  can  not 
be  definitely  ascertained. 

II.  THE  NEURALGIAS 

Neuralgia  is  used  to  denote  a  pain,  appearing  paroxysmally  and  spread- 
ing along  the  course  of  a  peripheral  nerve.  That  it  is  a  matter  of  an  anatom- 
ically limited  disease  of  the  peripheral  nervous  system,  cannot  be  doubted, 
since  it  appears  in  such  a  localized  manner. 

The  pathologic-anatomic  fundamental  elements  are  by  no  means  defi- 
nitely known.  It  may  be  assumed  that  it  is  a  matter  of  irritating  in- 
fluences affecting  the  nerve  in  some  point  or  other  of  its  course.  In  some 
forms,  indeed,  as  we  shall  see  below,  pathologic  changes  of  the  nerve  or  in  its 
vicinity  are  known,  which  may  cause  the  irritation.  How  and  when  the 
characteristic  attacks  take  place  after  the  nerve  is  affected  by  an  apparently 
permanent  injury,  is  still  undetermined. 

The  nerve  trunks  themselves,  as  seen  under  the  microscope,  are  in  many 
cases  entirely  normal.  The  slight  diffuse  neuritic  changes  which  occur  in 
some  cases,  are  probably  only  in  exceptional  instances  related  directly  to 
neuralgia.  As  a  rule,  neuritis  does  not  manifest  any  genuine  neuralgic 
symptoms.  On  the  other  hand,  slight  neuritic  symptoms  may  often  appear, 
even  clinically,  in  neuralgia. 

^Etiology 

In  many  cases  neuralgia  follows  infectious  diseases.  Probably  the  most 
frequent  one  in  Germany  is  that  which  appears  in  the  area  of  the  su- 
praorbital nerve  during  or  after  influenza.  In  persons  who  have  suffered 
from  malaria,  attacks  of  neuralgia  may  take  place  at  the  same  typical  in- 
tervals at  which  the  attacks  of  fever  appeared  (masked  malaria) .  Second- 
ary and  tertiary  syphilis  may  lead  to  neuralgia;  in  the  tertiary  form  it  is 
usually  produced  by  perineuritic  or  paraneuritic  gummatous  processes. 

Now  and  then  neuralgia,  especially  the  form  of  it  accompanying  herpes 
zoster,  appears  under  a  clinical  picture  of  an  independent  infectious  disease, 
and  even,  according  to  some  reports,  as  small  endemics. 

Toxic  processes  do  not  play  an  important  part  in  neuralgia.  Very 
prominent,  however,  is  the  predisposing  significance  of  metabolic  diseases, 
foremost  among  which  is  diabetes  mellitus. 

Colds  are  frequently  given  as  causes  of  neuralgia.  It  is  especially 
difficult  then  not  to  recognize  such  connection  between  the  two,  when  that 
part  of  the  body  which  was  affected  by  the  cold  becomes  the  seat  of  neu- 
ralgia (sciatica  after  sitting  on  the  cold  floor,  etc.) . 

Of  special  interest  are  those  cases  in  which  a  chronic  irritation  of  a  certain 
part  of  the  nerve  trunk  may  be  directly  proven.     We  mention  tumors  in  the 


NEURALGIA  177 

broadest  sense  of  the  word,  osteitis  and  periostitis,  bone  fragments,  scars  and 
inflammatory  processes  in  the  neighborhood  of  the  nerve.  Tumors  of  the 
nerve  itself  may  be  disclosed  by  true  neuralgia,  especially  in  the  early  stage. 
Diseases  in  the  area  of  the  nerve  roots,  the  vertebrae  and  meninges  must  be 
considered. 

The  pathogenic  irritation  may  proceed  also  from  the  peripheral  extension 
area  of  the  nerve.  Various  inflammatory  processes,  cicatrices  and  many 
painful  alterations  of  a  chronic  nature  may  occur.  In  amputation  neuromata 
the  patient  often  imagines  that  he  feels  the  pain  they  occasion  in  the  part  that 
has  been  removed. 

According  to  an  old  theory  neuralgia  may  be  excited  reflexly  from  a 
distance.  Thus  it  is  said  that  neuralgia  may  develop  in  a  distant  nerve  area 
from  the  intestines  because  of  intestinal  worms;  from  female  genital  organs 
because  of  a  reflexio  uteri,  etc.     This,  however,  is  very  questionable. 

Furthermore  transient  mechanical  irritations  of  a  nerve  may  result  in  a 
neuralgic  condition,  though  the  pathologic  connection  can  not  be  explained 
in  detail.  To  this  class  belong  neuralgia  after  contusion  of  the  affected 
part  of  the  body  and  postpartum  sciatica. 

Finally,  severe  functional  strain  upon  some  parts  plays  a  certain  role.  So, 
a  supraorbital  neuralgia  is  often  seen  after  any  work  in  which  the  eyes  were 
strained  and  a  long  continued  one  sided  strain  upon  one  leg  will  result  in  a 
sciatica  of  that  leg. 

Lastly  we  mention  that  general  debility  seems  to  develop  a  tendency  to 
neuralgia. 

Neuralgias  are  distinguished  as  symptomatic  and  idiopathic.  All  those 
cases  in  which  a  local  or  general  fundamental  disease  may  be  found  are  called 
symptomatic.  As  long  as  it  is  impossible  to  find  such  disease,  the  neuralgia 
is  called  idiopathic.  It  will  be  noted  that  this  classification  is  not  based  on 
any  important  internal  difference,  and  is  really  worthless.  Strictly  speaking, 
neuralgia  is  always  only  a  symptom. 

The  practical  result  of  this,  as  we  shall  point  out  also  later,  is  the  necessity 
of  looking  carefully  for  a  palpable  cause  in  every  case. 

Such  a  conception  also  makes  it  unnecessary  to  separate  from  neuralgia 
pains  of  a  true  neuralgic  character  manifesting  themselves  in  other  organic 
nerve  diseases,  such  as  basal  cerebral  tumors,  meningitic  processes. 

Clinical  Picture 

As  a  rule,  the  attack  of  pain  is  preceded  by  a  brief  premonitory  parses- 
thesia.  It  very  soon  attains  its  highest  point.  Pains  have  been  described  as 
splitting,  burning,  boring,  cutting  and  pricking,  and  very  frequently  they  are 
intolerably  violent.  Asked  for  the  seat  of  the  pain,  the  patient  will  often 
indicate  with  his  hand  or  finger  the  exact  course  of  the  diseased  nerve.  In 
grave  or  protracted  neuralgia,  an  irradiation  frequently  takes  place,  so  that 


178  DISEASES  OF  THE  PERIPHERAL  NERVES 

the  whole  vicinity  of  the  nerve  begins  to  pain  also.  Neuralgia  of  one  nerve 
may  be  accompanied  by  pains  in  its  secondary  branches  and  its  neighboring 
nerves. 

The  attack  may  pass  away  in  a  few  minutes  or  last  for  hours. 

The  attack  sets  in  either  spontaneously  or  is  excited  by  certain  external 
causes,  among  which  are  various  sensory  irritations,  particularly  those  that 
affect  the  diseased  area,  as  touch,  shock,  draughts  of  air,  thus  movements  and 
exertions  of  the  diseased  area,  for  instance  eating  in  trigeminal  neuralgia, 
walking  in  sciatica,  and  finally  coughing,  pressure,  mental  excitement  and 
changes  in  the  weather. 

Especially  in  chronic  cases,  and  most  frequently  in  sciatica,  slight  pains 
are  often  either  constant  or  nearly  constant,  and  the  attacks  appear  then  as 
temporary  more  or  less  pronounced  exacerbations. 

Of  foremost  diagnostic  importance  among  other  signs  of  the  disease  are 
the  so-called  pressure  points  (Valleix,  1852).  These  limited  points  of  exceed- 
ing susceptibility  to  pressure  are  found  in  places  where  the  nerve  has  been 
slightly  irritated  mechanically,  where  it  rests  exposed  upon  a  hard  founda- 
tion, where  it  emerges  from  bony  canals  or  where  it  pierces  a  fascia.  The 
tenderness  to  pressure  persists  usually  in  the  intervals  between  the  attacks; 
rarely  during  the  latter  alone.  It  is  said  that  it  may  even  be  lacking  entirely. 
Slight  pressure  on  the  pressure  point  is  liable  to  excite  an  attack,  while  strong 
pressure  is  often  soothing.  Less  often  the  entire  course  of  the  nerve  is 
tender  to  pressure.  Trousseau  regarded  as  very  important  the  regular  sus- 
ceptibility to  pressure  of  certain  spinous  processes  in  different  neuralgias. 

The  disease  is  further  accompanied  by  vasomotor,  secretory  and  trophic 
disorders.  Reddening  and  a  slight  swelling  of  the  skin  in  the  area  of  the 
pain  is  frequently  seen  during  the  attack  and  finally  may  become  habitual. 
More  extensive  cutaneous  changes  are  often  occasioned  by  mechanical 
mistreatment,  to  which  many  patients  expose  the  affected  parts  during  the 
attack.  Occasionally  hypersecretion  of  glands  situated  in  the  area  of  the 
injured  nerve,  especially  a  surplus  flow  of  tears  and  saliva  in  neuralgia  of  the 
fifth  nerve  are  seen.  Among  trophic  disorders  anomalies  of  hair-growth 
occur.  By  far  the  most  important  is  herpes  zoster,  which  will  be  discussed 
briefly  in  a  supplement  to  this  chapter. 

In  the  motor  area,  tonic  tension  and  clonic  spasms  of  the  muscles  in  the 
affected  parts  are  sometimes  seen  during  the  attack  of  neuralgia.  A  tic 
convulsif  may  combine  with  the  "tic  douloureux,"  in  trifacial  neuralgia. 
The  great  care  which  patients  bestow  upon  the  affected  member,  occasions 
a  kind  of  pseudo-paresis.  The  muscles  of  the  affected  area  become  more 
or  less  wasted  in  all  graver  and  more  protracted  cases.  As  a  rule,  however, 
true  pareses  or  paralyses  do  not  occur,  except  in  a  minority  of  cases  in  which 
a  local  progressive  process  forms  the  basis  of  neuralgia,  which  in  time  leads 
to  destruction  of  the  motor  nerve  fibres. 


NEURALGIA  179 

Sensory  disorders  are  frequently  represented  by  hyperesthesias  of  the 
skin.  Slight  hyperesthesias  in  peripheral  neuritic  and  sometimes  in  radicular 
areas,  also  occur  often. 

Weakening  or  loss  of  a  reflex,  such  as  the  Achilles  tendon  reflex  in  sciatica, 
is  in  grave  cases  very  common. 

It  is  hardly  necessary  to  mention  that  the  general  condition  of  the  patient 
may  be  greatly  injured  by  the  disease. 

But  little  remains  to  be  added  concerning  the  special  conditions  of  a 
few  quite  important  single  neuralgias. 

Trifacial  {trigeminal)  neuralgia,  aside  from  sciatica,  is  by  far  the  most 
frequently  occurring  form.  Usually  only  one  branch,  the  first  in  most  cases, 
is  affected.  The  most  important  pressure  points  are  situated  at  the  supra- 
orbital foramen  for  the  first  branch,  at  the  infraorbital  and  zygomatico- 
facial foramina  for  the  second,  and  at  the  mental  foramen  for  the  third.  It 
is  probably  only  in  syphilitics  that  a  bilateral  neuralgia  of  the  auriculo- 
temporal occurs,  the  pain  area  of  which,  in  the  form  of  a  child's  comb, 
extends  across  the  crown  from  one  ear  to  the  other,  and  whose  pressure 
point  is  situated  on  the  zygomatic  arch  in  front  of  the  ear  (Seeligmueller). 
In  neuralgia  of  the  first  branch,  the  pain  usually  extends  along  the  ramus 
supraorbitalis.  In  the  area  of  the  second  branch  a  predilection  for  the  rami 
infraorbitalis  and  alveolaris,  next  to  the  zygomatic  nerve  is  shown,  in  the 
area  of  the  third  branch,  for  the  ramus  alveolaris  inf. 

Among  the  causes,  the  affections  in  the  extension  area  of  the  nerve,  such 
as  diseases  of  the  nose  and  its  cavities  and  especially  of  the  teeth,  play  a 
special  part  particularly  in  trifacial  neuralgia.  Even  the  sharp  edge  of  the 
shrunken  alveolar  processes  in  persons  who  have  lost  their  teeth  may  excite 
a  neuralgia. 

Hence  the  advice  of  a  dentist  rarely  can  be  dispensed  with.  A  random 
extraction  of  sound  teeth,  such  as  has  been  observed  occasionally,  is  of 
course  wholly  inadvisable. 

If  in  herpes  zoster  trigemini  the  cornea  is  affected,  the  aid  of  an  oculist 
is  to  be  sought. 

In  occipital  neuralgia,  which  chiefly  affects  the  major  occipital  nerve,  and 
which  belongs  to  the  frequently  bilaterally  occurring  neuralgias,  tumors  of 
the  posterior  cranial  fossa  and  affections  of  the  uppermost  vertebrae  are 
aetiologically  to  be  considered.  The  main  pressure  point  is  situated  on  the 
linea  nuche  sup.  at  the  point  where  the  nerve  comes  to  the  surface  through  a 
hiatus  in  the  muscle. 

True  brachial  neuralgias  are  very  rare.  They  are  most  likely  to  occur 
(either  uni-  or  bilaterally)  in  affections  of  the  meninges  or  vertebras  and  in 
tumors  in  the  root  or  plexus  area.  In  an  etiological  search  cervical  ribs 
must  also  be  sought  for. 

Intercostal  Neuralgia. — Among  special  etiological  factors  are  affections  of 


180  DISEASES  OF  THE  PERIPHERAL  XERVES 

the  ribs,  various  intra-thoracic  diseases,  space  contracting  processes  such  as 
aneurisms  and  severe  scoliosis,  although,  of  course,  the  pains  in  the  chest 
occasioned  by  all  these  diseases  are  only  now  and  then  of  a  truly  neuralgic 
character.  The  pressure  points  are  situated  in  the  intercostal  space  near 
the  vertebrae,  in  the  mid-axillary  line  and  near  the  sternum. 

A  neuralgic  affection  of  the  nerves  of  the  mammary  glands  which  appears 
especially  after  trauma  during  pregnancy  and  lactation,  is  designated  as 
mastodynia  (irritable  breast,  Astley  Cooper). 

Neuralgias  of  the  lumbar  plexus  nerves,  neuralgia  lumbo-abdominalis, 
cruralis,  etc.,  are  very  rare. 

Sciatic  Neuralgia  (Ischiadica). — Sciatica,  the  malum  Cotunnii, 
(Cotugno,  1764)  is  the  most  frequent  form  of  neuralgia.  Among  the  special 
causes  are  diseases  of  the  os  sacrum  and  pelvic  bones,  tumors  and  exudations 
of  the  pelvis,  displacements  of  the  uterus,  sciatic  hernia  and  chronic  consti- 
pation. A  manual  examination  of  the  pelvic  organs,  through  the  rectum, 
should  never  be  omitted  in  any  case  of  sciatica. 

In  bilateral  sciatica  there  is  always  a  suspicion  of  a  meningeal  or  vertebral 
affection.     Next  to  that,  it  is  probably  most  likely  to  occur  in  diabetes. 

Sciatic  neuralgia  may  be  restricted  to  single  branches,  like  the  posterior 
cutaneous. 

The  pressure  points  are  situated  at  the  great  sacro-sciatic  foramen,  in  the 
gluteal  crease,  in  the  popliteal  space,  in  the  middle  of  the  calf,  at  the  head 
of  the  fibula,  behind  the  ankle,  sometimes  also  laterally  from  the  tubercles 
of  the  os  sacrum,  somewhat  below  the  middle  of  the  crest  of  the  ilium  and 
in  other  places. 

Important  for  the  diagnosis  is  the  pain  when  stretching  the  nerve. 
Flexion  of  the  hip  joint  when  the  knee  is  in  extension  excites  violent  pains, 
while  no  pains  are  occasioned  if  the  same  movement  is  executed  with  the 
knee  flexed  (phenomenon  of  Lasegue). 

The  patient  is  apt  to  carefully  avoid  all  movements  which  might  produce 
such  stretching  and  he  likewise  avoids  exposing  the  back  part  of  the  leg, 
and  through  it  the  nerve,  to  any  pressure.  Most  patients  keep  the  leg 
slightly  flexed  at  hip  and  knee  both  when  lying  and  walking  and  rotate  it 
somewhat  outward.  When  standing  or  walking  they  throw  the  weight  of 
the  body  on  the  healthy  leg.  The  trunk  is  slightly  lowered  towards  the 
healthy  side  (sciatic  scoliosis).  In  some  rare  cases,  however,  a  slight 
deviation  of  the  spinal  column  in  the  opposite  direction  takes  place.  The 
origin  of  the  scoliosis  is  still  very  doubtful  (Figs.  40  and  41). 

The  reflexes  of  the  affected  leg  are  not  infrequently  slightly  increased. 
It  has  already  been  mentioned  that  sometimes  they  are  also  weakened. 
Occasionally  a  peculiar  kind  of  '''pseudo-Babinski"  reflex  is  found,  whose 
nature  has  not  yet  been  explained. 

The  diagnosis  of  a  typical  neuralgia  is  easy,  if  strict  regard  be  given 


DIAGNOSIS  OF  NEURALGIA 


181 


to  the  main  symptoms,  which  have  been  mentioned  in  the  definition,  the 
anatomic  extension  and  the  paroxysmal  character  and  course  of  the  pain. 
Difficulties  ought  to  arise  only  in  atypical  forms. 

When  the  course  as  to  the  paroxysms  is  not  clearly  pronounced,  as 
occurs  in  cases  of  long  standing  and  especially  also  in  sciatica,  the  other 
symptoms,  particularly  the  localization  of  pain  and  perhaps  also  the  anam- 


Fig.  40.  Fig.  41. 

Figs.  40  and  41. — Sciatica  at  the  left  side.     Characteristic  posture  of  the  leg,  scoliosis  ischiadica. 

(Leipsic  Medical  Clinic.) 

nestic  statement  that  characteristic  attacks  occurred  before,  will  frequently 
permit  of  a  correct  diagnosis. 

Where  the  localization  in  the  course  of  a  certain  peripheral  nerve  cannot 
be  authenticated,  especially  in  neuralgoid  conditions,  in  which  the  pain  is 
confined  to  a  very  narrow  area,  it  will  as  a  rule,  be  impossible  to  come  to 
any  definite  conclusion.  This  is  true  for  instance  of  the  so-called  joint 
neuralgia,  achillodynia,  mastodynia,  coccygodinia  and  visceral  neuralgias. 

In  all  doubtful  cases  it  should  be  ascertained  whether  other,  especially 
inflammatory  affections,  do  not  cause  the  existing  pain.     As  we  have  seen, 


1 82  DISEASES  OF  THE  PERIPHERAL  NERVES 

the  existence  of  such  a  disease,  of  an  inflammation,  or  of  a  tumor  would  not 
necessarily  exclude  the  existence  of  neuralgia.  The  latter  may  exist  inde- 
pendently of  such  affections,  but,  on  the  other  hand,  may  not  only  be 
unfavorably  influenced  by  them,  but  even  caused  by  them.  It  will  therefore 
always  be  a  question  of  whether  criteria  of  neuralgic  pains  are  present  or 
whether  the  disorders  correspond  simply  in  nature  and  course  to  the  other 
disease  which  has  been  found. 

We  shall  now  mention  a  number  of  diseases,  which  as  experience  has 
taught  us,  are  frequently,  with  but  a  superficial  examination,  mistaken  for 
neuralgia.  Thus  neuralgic  pains  are  easily  confounded  with  the  acute  and 
radiating  pains  in  diseases  of  large  joints  of  the  extremities,  especially  of 
the  hip  and  shoulder  joints,  in  hereditary  deformities  such  as  flat-foot,  in 
affections  of  the  arteries  and  veins  (intermittent  claudication,  varicose  veins), 
and  in  occupation  neuroses.  The  so-called  lymphangitis  rheumatica 
(Wilms1)  must  also  be  considered. 

Catarrh  of  the  frontal  sinus  and  even  common  attacks  of  migraine  have 
occasioned  a  wrong  diagnosis  by  being  mistaken  for  trifacial  neuralgias  and 
beginning  pleurisy  has  been  confounded  with  intercostal  neuralgia. 

Of  a  more  general,  differential  diagnostic  significance  are  muscular 
rheumatism,  tabetic  pains,  spasms,  neuritis,  those  reflex  pains  and  hyper- 
esthesias in  visceral  affections  which  have  been  investigated  mainly  by 
Head,  to  which  perhaps  belong  the  pains  in  angina  pectoris,  and  psychogenic 
pains. 

In  all  these  painful  diseases  the  characteristic  signs  of  neuralgia,  aside 
from  those  rarer  cases  which  are  combined  with  a  true  neuralgia,  are  of 
course  lacking.     We  can  not  here  deal  with  their  positive  symptoms. 

The  differential  diagnostic  significance  of  psychogenic  pains  is  con- 
siderably enhanced  by  the  fact  that  in  some  cases,  particularly  in  individuals 
with  a  nervous  predisposition  and  in  those  who  because  of  injuries  claim 
insurance  money,  they  may  add  themselves  to  true  neuralgia  and  strongly 
resemble  it.  At  any  rate,  it  may  be  seen  in  such  cases  how  the  type  of  the 
neuralgic  picture  becomes  more  and  more  obliterated,  while  the  signs  of 
the  "psychalgia"  (Oppenheim),  the  determination  of  the  form  and  course 
of  the  disorders  by  certain  ideas,  the  dependence  upon  attention  and  expecta- 
tion, becomes  more  and  more  pronounced  and  exclusive.  Perhaps  in  those 
cases  of  neuralgia,  which  ostensibly  have  been  cured  by  hypnotism,  it  was  a 
matter  of  such  psychogenic  residual  images. 

Once  the  presence  of  neuralgia  has  been  conclusively  determined,  the 
investigation  should  continue  as  to  the  aetiology.  The  general  condition  of 
the  body  should  be  carefully  considered;  one  should  search  for  infectious 
and  toxic  conditions  and  for  metabolic  disturbances.  Next,  those  parts 
through  which  the  diseased  nerve  takes  its  course,  should  be  closely  scruti- 

1  Miinchener  med.  Wochenschr.,  1906,  page  1595. 


PROGNOSIS  AND  THERAPY  OF  NEURALGIA  183 

nized,  to  see  whether  any  alterations  exist  which  might  sustain  or  produce 
the  irritated  condition  of  the  nerve.  Above  all  it  should  be  discovered 
whether  some  grave,  progressive,  local  disease  is  not  hidden  behind  the 
neuralgia,  such  as  a  malignant  neoplasm  or  something  of  the  sort.  Thus, 
for  example,  a  tubercular  meningitis  may  begin  under  the  innoxious  picture 
of  a  sciatica.  Bilaterality,  which  is  not  infrequent,  rise  in  temperature,  the 
proof  of  other  tubercular  diseases  or  of  a  tubercular  diathesis,  may  early 
confirm  one's  suspicion. 

The  prognosis  should  always  be  made  with  caution,  even  in  those 
cases  where  it  is  believed  that  a  graver  fundamental  disease  does  not  exist. 
The  prospects  are  comparatively  favorable  in  many  post-infectious  neu- 
ralgias. A  neuralgia  following  influenza  may  recover  in  a  few  days.  Dia- 
betic neuralgia  often  disappears  after  one  succeeds  in  removing  or  at  least 
considerably  diminishing  the  glycosuria.  In  the  majority  of  cases  a  neu- 
ralgic affection  lasts  a  number  of  weeks  or  a  few  months.  Even  its  con- 
tinuation for  several  years  is  by  no  means  uncommon. 

Old  age,  a  poor  general  condition  of  the  patient,  a  long  course  and 
particularly  the  severity  of  the  disease  all  darken  the  prospects  for  recovery. 
In  many  cases  the  tendency  to  relapse  is  quite  marked. 

Therapy. — On  account  of  the  many  sided  setiological  relations  of 
neuralgia  great  care  is  required  in  its  treatment.  The  general  condition 
should  be  improved  as  far  as  possible.  Any  existing  morbid  disorders, 
particularly  those,  of  course,  that  from  experience  are  known  to  have  been 
factors  in  the  origin  of  neuralgia,  should  be  taken  up  for  treatment.  After 
that  one  should  endeavor  to  remove  the  local  causes. 

Abstinence  from  alcohol  is  almost  invariably  to  be  advised;  the  use  or 
non-use  of  coffee  or  tea  must  be  decided  upon  individual  grounds  in  each 
case.  Experienced  authors  warn  against  a  one  sided  diet,  unless  it  is  very 
definitely  indicated.  Regulating  the  bowels  is  of  great  importance  in  all 
cases;  in  a  few  rare  cases  it  has  actually  direct  curative  value. 

Particularly  in  recent  cases  it  is  advisable  to  keep  the  affected  parts  in 
a  condition  of  absolute  rest  for  some  time.  Recent  cases  of  sciatica  neces- 
sitate confinement  to  bed  for  two  or  three  weeks,  longer  if  the  condition  of 
the  patient  requires  it.  It  will  sometimes  be  advisable,  to  secure  rest  for  the 
diseased  parts  in  a  way  most  comfortable  to  the  patient  by  means  of  splints 
or  by  other  fixation  methods. 

From  the  abundance  of  various  remedies  we  have  selected  the  following 
important  ones,  which  have  stood  the  test  of  time. 

Among  the  medicinal  agents,  quinine,  iron,  mercury  and  the  iodides  play 
their  well  known  role  in  the  specific  setiological  treatment  of  malaria,  anemia, 
syphilis  and  lead  poisoning. 

A  general  treatment  with  tonics,  particularly  arsenic  and  strychnine,  and 
with  the  ubiquitously  tried  iodides  is  believed  to  be  useful  in  many  cases. 


1 84  DISEASES  OF  THE  PERIPHERAL  NERVES 

The  antineuralgics  occupy  a  foremost  position.  In  recent  cases,  a 
regular  use  of  several  (say  about  3)  doses  daily  is  advised,  afterwards  they 
may  be  kept  in  reserve  to  be  used  when  necessary.  Of  the  great  number  of 
these  agents  we  mention  antipyrine  (0.5  to  1.0  per  dose);  phenacetine  (0.5); 
antifebrin  (0.25);  migrainine  or  antipyreticum  compos,  Riedel  (1.0);  pyr- 
amidon  (0.3) ;  lactophenin  (0.5);  aspirin  (0.5  to  1);  quinine  sulfat.  or  muriat. 
(0.5).  When  the  single  preparations  do  not  prove  successful,  mixtures  may 
often  prove  of  value,  such  as  antipyrine  0.5,  phenacetine  antifebrin  aa  0.25, 
1/2  powder  for  one  dose.  Combinations  with  bromine  and  with  codeine 
are  to  be  recommended.  As  another  agent  adapted  to  alleviate  pain,  we 
mention  butylchloral.  We  caution  against  the  use  of  morphine,  in  view 
of  its  uncertain  action,  and  the  danger  of  morphinism,  since  the  duration  of 
the  disease  can  not  be  foretold.  In  extreme  cases  single  doses  of  mor- 
phine or  its  derivatives  (heroin,  etc.),  will  have  to  be  given,  just  as  one 
will  sometimes  have  to  resort  to  a  soporific  (chloral  hydrate).  In  some 
exceptional  cases,  atropine  is  found  to  be  soothing  where  morphine  has 
failed. 

Hydro-  and  Thermo -therapy. — Of  the  general  applications  in  recent, 
not  very  sensitive,  cases  warm  baths  of  half  an  hour's  duration,  or  hot  baths 
for  a  shorter  time,  taken  several  times  a  week  should  be  considered,  also  the 
more  energetic,  diaphoretic  hot  air  baths  in  bed  (they  may  be  taken  even  by 
the  most  seriously  affected  patients),  by  means  of  such  apparatus  as  that  of 
Hilzinger,  of  1200  to  1400  F.,  and  about  1/4  to  1/2  hour  length.  Later,  espe- 
cially as  after-treatment,  thermal  baths,  carbonic  acid  and  brine  baths  may 
be  recommended. 

Local  thermic  procedures  are  usually  applied  upon  the  painful  places 
and  preference  will  almost  always  have  to  be  given  to  warm  or  hot  applica- 
tions. Moist  or  dry,  warm  or  hot  bandages,  cataplasms,  thermophores  or 
similar  applications,  packing  in  peat  or  mud,  sand  baths,  hot  air  baths,  most 
conveniently  in  Bier's  apparatus,  radiations  from  photo-therapeutic  appara- 
tus are  successfully  prescribed,  those  most  convenient  to  the  patient  in  the 
beginning,  the  others  in  the  more  advanced  stages  of  the  affection. 

From  the  group  of  the  so-called  derivative  agents  which  have  stood  the 
test  of  experience,  we  recommend  especially  dry  cupping  glasses,  to  be  used 
even  in  recent  cases  and  applied  daily  along  the  course  of  the  affected  nerve 
in  number  corresponding  to  the  extension  of  the  painful  area.  Embrocations 
which  irritate  the  skin,  tincture  of  iodine  applied  with  a  brush,  plasters  which 
produce  cutaneous  irritations  and  which  may  be  left  on  for  several  days 
(emplastr.  cantharid.  perpet.  and  empl.  oxycroceum,  etc.),  applications  of 
menthol  spirits  and  menthol  oil  under  covering  compresses,  perhaps  some- 
times even  real  vesicants  (emplastr.  cantharid.  ordin.)  may  all  be  used  to 
alleviate  the  pain.  Blood  letting,  applications  of  moxae,  etc.,  should  be 
resorted  to  but  seldom. 


THERAPY  OF  NEURALGIA  185 

The  chief  method  of  electro-therapy  is  the  stabile  anodic  galvanization 
of  the  main  points  of  the  pain. 

A  button  shaped  electrode  (surface  5  to  10  qcm.)  is  placed  on  the  pressure 
point;  a  larger  one,  the  cathode,  is  placed  as  the  dispersing  electrode  on  any 
point  in  the  healthy  area,  as  for  instance,  in  sciatica  in  the  region  where  the 
nerve  originates  (os  sacrum  region).  In  order  to  attain  success  it  will  be 
necessary,  when  beginning  and  ending  the  treatment,  to  increase  and 
diminish  the  current  very  carefully  and  to  avoid  all  irritating  influences. 
Weak  currents  of  about  2  ma.,  when  the  nerve  is  situated  deeper,  3  to  4  ma., 
should  be  used.  Seances  to  last  from  2  to  5  minutes.  Occasionally  more 
protracted  applications  may  be  tried.  At  the  same  sitting  various  pressure 
points,  each  for  about  2  minutes,  may  be  treated  successively.  Galvanic 
treatment  is  given  from  3  to  7  times  a  week;  in  exceptional  cases  even  twice 
daily.  If  applied  correctly  the  result  will  not  infrequently  prove  a  direct 
palliation  of  the  troubles. 

Besides  this  preeminently  successful  method,  a  great  number  of  others 
have  been  recommended.  Particularly  in  inveterate  cases,  for  instance  of 
sciatica,  it  may  be  expedient  to  place  both  electrodes  (about  10  qcm.  in  size 
each)  proximally  and  distally  on  the  affected  nerve  and  to  let  somewhat 
stronger  currents  (about  5  to  6  ma.)  flow  through  it  for  about  5  minutes. 
During  this  treatment  the  position  of  the  electrodes  may  be  changed.  It 
seems  to  make  no  difference  whether  the  "ascending"  (cathode  proximal) 
or  the  "descending"  current  is  used. 

Faradism  is  not  as  valuable  as  galvanization.  In  the  lighter  cases. of 
long  standing  faradization  of  the  skin  and  muscles  of  the  affected  area  is 
sometimes  beneficial.  The  treatment  of  painful  places  by  means  of  the 
faradic  brush  should  be  tried  only  in  exceptional  cases. 

Mechano-therapy. — In  inveterate  cases,  particularly  of  sciatica,  mas- 
sage of  the  affected  parts  is  exceedingly  serviceable.  Rubbing  and  knead- 
ing may  be  followed  by  vibratory  massage  of  the  affected  nerve  along  its 
entire  exposed  course.  In  recent  cases  massage  almost  invariably  proves 
a  failure. 

Stretching  the  nerve,  which  today  is  probably  used  only  in  the  form  of  so- 
called  bloodless  stretching,  may  be  appropriately  combined  with  massage. 
For  example,  in  sciatica  after  the  leg  has  been  massaged  and  is  in  extension 
at  the  knee  it  should  be  lifted  from  its  support  very  slowly,  so  as  to  occasion  as 
little  pain  as  possible  and  then  be  flexed  in  the  hip  joint  more  and  more. 
Avoiding  all  rough  manipulations  one  should  proceed  as  far  as  the  patient 
can  bear  it,  then  keep  the  leg  in  that  position  for  1  to  2  minutes,  and  then 
perhaps  after  a  final,  stronger  stretching,  let  the  leg  down  slowly.  Manual 
stretchings  of  aching  cutaneous  areas  are  recommended  also,  and  have 
undoubtedly  been  used  in  some  cases,  in  which  they  afforded  an  instantaneous 
palliation  of  pain  (Naegeli). 


1 86  DISEASES  OF  THE  PERIPHERAL  NERVES 

Climatic  therapy  is  of  no  considerable  importance.  In  general,  very 
changeable,  irritating,  cold  or  moist  climates  should  be  avoided. 

Of  the  many  methods  of  injection  of  different  liquids  into  the  nerve  trunk 
or  its  immediate  vicinity  we  recommend  chiefly  a  trial  of  the  method  given  by 
Lange,  which  is  almost  entirely  painless.  It  consists  in  a  paraneuritic 
injection,  repeated  when  necessary,  of  rather  large  quantities  (about  ioo  ccm. 
in  sciatica)  of  a  solution  of  eucaine-/?  in  a  physiological  solution  of  cooking 
salt  (cf.  Munchener  med.  Wochenschrift,  1904,  No.  52,  page  2325).  This 
causes  a  rapid  palliation  and  even  stopping  of  the  pain,  and  in  some  cases 
leads  to  a  rapid  and  permanent  recovery. 

Among  the  surgical  methods,  which  are  discussed  more  fully  elsewhere 
in  this  volume,  neurolysis,  releasing  the  nerve  from  its  irritating  surroundings 
and  neurinsarcoclesis  may  be  ranked  among  the  treatments  removing  the 
cause.  The  mutilating  operations  of  neurotomy  and  neurectomy,  of  neur- 
exairesis  and  of  extirpation  of  ganglia,  can  be  advised  only  in  those  cases 
in  which  the  resources  of  internal  medicine  have  been  exhausted. 
An  operation  should  not  be  performed  before  careful  galvanic  treatment, 
which  even  in  cases  of  old  standing  often  rewards  us  with  surprising  results, 
has  been  applied  by  an  expert.  Even  surgical  methods  do  not  insure  a 
permanent  cure. 

SUPPLEMENT 

Herpes  Zoster 

Herpes  zoster  consists  of  an  eruption  accompanied  by  pain.  Quite 
rapidly  there  develop  one  after  another  groups  of  vesicles  on  a  reddening 
surface.  These  localized  groups  have  a  highly  characteristic  lengthwise 
formation  in  a  stripe-like  area,  corresponding  to  the  innervation  area  of  a 
certain  nerve  root,  or  sometimes  of  a  peripheral  branch.  The  affection  which 
is  almost  invariably  unilateral,  most  frequently  occupies  the  area  of  an  inter- 
costal nerve,  a  somewhat  beltlike  region  on  one  side  of  the  thorax,  whence 
its  name.  Next  to  this,  the  trigeminal  area  is  most  often  affected.  Some- 
times the  vesicles  disappear  after  a  few  days,  leaving  either  brown  spots, 
which  later  disappear,  or,  if  the  efflorescences  were  hemorrhagic  or  gangren- 
ous, white  scars  typically  grouped. 

Anatomically,  with  the  greatest  regularity  a  disease  of  the  peripheral 
sensory  neuron,  especially  of  the  spinal  ganglia,  but  not  infrequently  also  of 
the  peripheral  nerves,  and  frequently  true  inflammatory  changes,  are  found. 
The  real  relation  between  the  cutaneous  affections  and  the  nerve  alterations 
is  entirely  obscure. 

Frequently,  though  by  no  means  invariably,  signs  of  a  greater  suspension 
of  function  and  anaesthesia  occur  during  herpes  zoster.     Motor  paralyses,  like 


HERPES  ZOSTER 


187 


facial  and  eye-muscle  paralyses  in  herpes  zoster  trigemini,  are  symptoms  that 
rarely  occur. 

Many  traits  of  which  we  mention  only  the  frequent  initial  fever,  the  occur- 
rence of  epidemics,  and  the  immunity  which  from  the  rareness  of  relapses 
may  be  supposed  to  exist,  all  indicate  that  in  numerous  cases  a  peculiar 
infectious  process  forms  the  foundation  for  this  clinical  picture.  Among 
the  causes  of  herpes  zoster,  there  are  a  number  of  factors  which  we  have  also 
found  in  the  aetiology  of  neuralgia,  namely :  other  infectious  diseases,  affections 
of  the  vertebrae,  trauma,  etc. 


Fig.  42.  Fig.  43. 

Figs.  42  and  43. — Herpes  zoster.  Fig.  42,  in  the  region  of  one  of  the  middle  thoracic  nerves. 
Fig.  43,  in  the  region  of  the  second  thoracic  nerve.  On  the  shoulder  at  the  right  a  birthmark, 
(Leipsic  Medical  Clinic.) 

Herpes  zoster  is  closely  related  to  carbon  oxide  and  arsenical  poisoning. 
Occasionally  herpes  zoster  manifests  itself  in  connection  with  diseases  of 
internal  organs,  by  appearing  in  a  neighboring  cutaneous  zone,  for  instance 
in  inflammations  of  the  costal  pleura.  The  connection  between  the  two  has 
not  yet  been  explained.  In  many  cases  of  herpes  zoster,  no  definite  aetiolog- 
ical  factors  can  be  found. 

The  relation  of  herpes  zoster  to  neuralgia  can  probably  be  summed  up 
thus:  that  they  stand  in  a  sort  of  co-ordinated  position  to  each  other.  The 
anatomic  changes  of  the  nerve  which  form  the  basis  of  the  cutaneous  affec- 
tion, in  many,  but  not  all  cases,  give  rise  to  neuralgia.     It  may  be  that  the 


1 88  DISEASES  OF  THE  PERIPHERAL  NERVES 

disorder  occasionally  is  not  accompanied  by  a  cutaneous  affection,  and  that 
it  becomes  apparent  only  as  a  result  of  a  neuralgia. 

Zoster  neuralgias  form  but  a  comparatively  small  percentage  of  all  the 
cases  of  neuralgia. 

Neuralgia  may  precede  herpes  zoster  for  some  time,  may  begin  in 
association  with  it,  or  may  follow  it. 

No  difficulty  is,  as  a  rule,  experienced  in  distinguishing  zoster  from  other 
forms  of  herpes.  In  treating  the  eruption  one  may  generally  confine  oneself 
to  a  light  protective  bandage,  some  dusting  powder,  etc. 

III.   THE  TUMORS  OF  THE  PERIPHERAL  NERVOUS  SYSTEM 

As  we  have  seen,  tumors  seizing  a  nerve  metastatically  or  by  spreading 
thence  from  a  neighboring  organ,  may  lead  to  its  neuralgic  affection  or 
paralysis. 

Neuromata,  the  tumors  emanating  primarily  from  the  peripheral  nerve, 
may  occasion  the  same  clinical  picture.  The  special  symptoms  of  these 
tumors  require  a  separate  discussion. 

It  is  relatively  rare  that  neuromata  emanate  from  the  nervous  tissue 
proper.  It  is  usually  a  matter  of  so-called  spurious  neuromata,  tumors  that 
emanate  from  the  connective  tissue  of  the  nerve  and  which  histologically 
appear  as  fibromata,  lipomata,  or  even  as  angiomata.  Some  tumors  may 
degenerate  and  become  malignant. 

We  shall  consider  first  those  forms  appearing  singly  or  at  any  rate 
confined  to  a  certain,  small  area  of  the  body.  They  develop  either  on  the  larger 
trunks  or  on  the  fine  terminal  ramifications,  particularly  in  the  subcutis. 
The  latter  group  includes  the  so-called  plexiform  (Ranken)  neuromata, 
which  are  almost  exclusively  of  merely  cosmetic  surgical  interest,  and  the 
small,  innoxious  nodules,  designated  as  tubercula  dolorosa.  These  are 
often  very  dense,  sometimes  angiomatously  livid  and  painful.  They  appear 
singly  or  in  small  groups  and  may  be  easily  removed  by  an  operation. 

The  neuromata  of  the  trunk  not  infrequently  lead  to  neuralgia  and 
paralysis.  Intra-cranial  neuromata  of  the  cranial  nerves,  as  they  appear, 
especially  on  the  auditory  and  trigeminal,  give  the  same  clinical  picture  as 
the  basal  brain  tumors. 

Neuromata  on  the  intra-vertebral  spinal  cord  roots  produce  the  symptoms 
of  extra-medullary  spinal  tumors. 

Besides  this  form  of  solitary  neuromata  there  exists  one  of  multiple 
neuromatosis.  The  most  frequent  form,  of  the  latter  is  the  general  one  of  the 
skin  and  subcutis.  Fibromata  or  lipomata,  softer  or  harder  nodules,  are 
generally  found,  sometimes  in  enormous  numbers,  distributed  over  the  whole 
surface  of  the  body.  The  neurofibromatosis  of  the  skin  is  designated  also 
as  Recklinghausen's  disease  (after  Recklinghausen  1882).     In  some  cases, 


NEUROFIBROMATOSIS  189 

however,  the  tumors  of  the  large  nerve  trunks,  of  the  cranial  nerves  and 
spinal  roots  appear  in  large  numbers.  Occasionally  numerous  neuromata 
are  found  in  the  skin  besides  the  single  or  several  centrally  situated  tumors  of 
this  nature,  and  the  determining  of  these  may  become  very  valuable  for  a 
correct  diagnosis  of  any  central  tumors  appearing  under  the  disease  picture 
of  a  spinal  or  brain  tumor.  At  any  rate,  the  most  varied  forms  and  local- 
ization of  neuromata  may  be  found  together  in  one  and  the  same  individual : 
besides  nodules  on  the  nerve  trunk,  appearing  in  a  wreath  like  formation, 
root  and  skin  neuromata,  multiple  tumors  in  the  deeper  part  of  the  body,  in 
the  muscles  and  the  intestines,  so  that  one  may  indeed  speak  of  a  general 
neuromatosis. 

The  disease  is  evidently  based  upon  a  congenital  predisposition.  It 
may  appear  even  very  early  or,  on  the  other  hand,  only  late  in  life.  It 
occurs  in  whole  families.  The  persons  affected  with  it  often  show  other 
signs  of  a  morbid  disposition  and  are  affected  with  deformities,  numerous 
naevi,  pigment  anomalies,  etc.,  and  show  a  neuropathic  diathesis. 

The  clinical  importance  of  the  disease  varies  with  the  number,  size  and 
location  of  the  tumors.  Especially  the  neurofibromatosis  of  the  skin  may 
be  almost  devoid  of  troublesome  features.  In  other  cases  they  may  become 
very  annoying  through  acute  pains.  Some  neuromata  situated  compara- 
tively indifferently,  may  become  dangerous  through  excessive  growth.  The 
danger  of  sarcomatous  degeneration  in  multiple  neuromatosis  and  in  soli- 
tary neuroma  of  the  trunk  is  rather  great. 

Aside  then  from  the  entirely  harmless,  or  nearly  so,  local  affection  of  the 
surface  of  the  body,  the  prognosis  is  very  uncertain.  There  are  cases  of  a 
mild  nature,  entirely  lacking  in  any  propensity  to  propagate,  but  there  are 
others  also  with  a  strong  tendency  to  growth  on  the  part  of  the  existing  tumors, 
and  a  disposition  to  form  more  and  more  new  ones.  Strange  to  say,  a  spon- 
taneous retrogression  of  single  tumors  has  also  been  reported. 

Surgical  treatment  should  be  used  if  seen  to  be  absolutely  necessary. 
When  advising  patients  one  should  always  remember,  however,  that  in  such 
cases  as  well  as  in  all  others,  mechanical  irritation  may  cause  the  trans- 
formation of  harmless  tumors  into  malignant  ones. 


Ill 

DISEASES  OF  THE  SPINAL  CORD 

i.  NORMAL  AND  PATHOLOGICAL  PHYSIOLOGY  OF  THE  SPINAL 

CORD 

BY 

MAX  ROTHMANN  (Berlin) 

The  spinal  cord  represents  phylogenetically  the  oldest  apparatus  of  the 
central  nervous  system.  In  the  lowest  vertebrate,  the  amphioxus,  it  repre- 
sents therefore,  since  the  brain  is  quite  rudimentary,  a  virtually  independent 
central  apparatus,  which  shows  normal  functions,  even  after  the  removal  of 
the  head-end.  In  the  ascending  scale  of  vertebrates,  the  dependence  of 
the  spinal  cord  on  the  various  higher  centers  of  the  brain  increases  continu- 
ously, and  special  investigation  is  needed  in  the  highest  mammals,  to  still 
find  individual  independent  functions  of  the  spinal  cord.  It  is  therefore 
readily  understood,  that  the  spinal  cord  in  building  up  its  own  centers,  and 
its  connections  with  the  brain,  furnishes  us  means  to  recognize  anatomical 
and  physiological  arrangements  peculiar  to  itself  in  each  and  every  species 
of  animal.  Comparative  anatomy  as  well  as  physiology,  therefore,  may 
transfer  only  with  the  greatest  caution,  the  facts  gleaned  from  one  species  of 
animal  to  another  however  nearly  related.  This  should  be  especially 
considered  in  making  use  of  the  result  of  animal  investigation  for  the  pur- 
poses of  human  pathology. 

The  spinal  cord  lies  in  the  vertebral  canal.  At  the  upper  end,  it  is 
rather  arbitrarily  bounded  by  the  exit  of  the  first  cervical  nerve  and  the  lower 
boundary  of  the  crossing  of  the  most  important  cerebro-spinal  tract,  the 
pyramidal  crossing.  At  the  lower  end,  in  adult  man,  it  is  far  from  reaching 
the  bottom  of  the  vertebral  canal,  but  as  a  rule  reaches  only  the  lower  part  of 
the  first  lumbar  vertebra.  In  lower  mammals  and  in  the  early  foetal  life  of 
man,  on  the  other  hand,  it  occupies  the  entire  cavity  of  the  lumbar  and  sacral 
canal.  While  the  spinal  cord  in  its  development  thus  remains  behind  the 
growth  of  the  vertebral  column,  it  is  replaced  in  the  lumbar  and  sacral 
vertebrae  by  a  delicate  thread,  the  filum  terminate. 

By  this  absence  of  the  spinal  cord  in  the  lower  part  of  the  vertebral  canal 
of  man,  lumbar  puncture  invented  by  Quincke,  at  the  level  of  the  lower  lumbar 
vertebrae,  has  been  made  possible  without  danger  of  lesion  to  the  spinal  cord. 

The  spinal  cord  is  enclosed  by  the  spinal  membranes.  On  the  outside 
lies  the  dura  mater,  a  comparatively  wide  sac,  which  at  the  lower  end  fuses 

190 


ANATOMY  OF  THE  SPINAL  CORD 


191 


with  the  filum  terminale.     On  the  inside  of  the  dura  lies  the  arachnoid 

which,  as  a  fine  network,  fills  the  space  between  the  dura  and  the  pia,  and 

contains  the  cerebro-spinal  fluid,  and  the  pia  mater,  lying  directly  upon  the 

surface  of  the  spinal  cord  and  sending  branches  into  its  fissures.     The  spinal 

cord  is  usually  divided  into  cervical,  dorsal, 

lumbar  and  sacral  portions  with  the  end 

in   the   conus   terminalis;   this  latter,  by 

gradual  attenuation,   goes  over  into  the 

filum    terminale.     To    this    corresponds 

the  division  of  the  spinal  cord  roots  into 

8  cervical  roots,  the  uppermost  of  which 

emerges  from  the  vertebral  canal  between 

the  occipital  bone  and  the  atlas,  whereas 

the  lowest  emerges  between  the  7  cervical 

and  the  first  dorsal  vertebrae,  into  12  dorsal, 

5  lumbar  and  5  sacral  roots,  the  lowest 

of  which  leaves  the  vertebral  canal  between 

the   os  sacrum  and  the  os  coccygis,  and 

the  coccygeal  nerve. 

The  above  mentioned  gradual  short- 
ening of  the  spinal  cord  in  relation  to 
the  vertebral  canal,  leads  to  a  displace- 
ment of  the  spinal  cord  segments  upwards, 
so  that  the  individual  spinal  cord  roots, 
in  order  to  reach  the  foramina  of  exit  des- 
tined for  them  between  the  vertebrae,  have 
to  course  some  distance  downwards.  The 
length  of  the  lumbar  and  especially  of  the 
sacral  roots,  is  considerable;  the  downward 
coursing  lumbar  and  sacral  roots  form, 
around  and  below  the  conus  terminalis, 
the  cauda  equina.  The  relation  between 
the  spinal  origin  of  the  individual  roots 
and  their  places  of  exit  from  the  inter- 
vertebral foramina,  which  is  of  the 
greatest  importance  for  spinal  cord  locali- 

0  r  r  ^  Fig.   44. — Scheme  of  location  of  the 

zation,  appears  clearly  in  Reid's  diagram  individual  spinal-cord  segments  in  the 
/-,-,.  N        T       ,,        j  ,  ,.  j.   ,,       vertebral  canal.     (After  Reid.) 

(.big.   44).     In  the  dorsal  portion  of  the 

cord,  the  entry  of  the  root  into  the  cord  lies  about  2  to  3  vertebrae  above 
the  corresponding  intervertebral  foramen  of  the  vertebral  canal,  but  in  the 
lumbar  and  sacral  roots  this  difference  increases  with  extraordinary  rapid- 
ity; so  that,  for  instance,  the  sacral  roots  all  have  their  spinal  origin  at  the 
height  of  the  twelfth  dorsal  and  first  lumbar  vertebrae. 


192  DISEASES  OF  THE  SPINAL  CORD 

In  the  spinal  cord,  there  are  two  rather  pronounced  enlargements,  corre- 
sponding to  the  origin  of  the  roots  of  the  extremities,  the  cervical  enlargement 
from  about  the  third  cervical  to  the  first  dorsal  vertebra  and  the  lumhar 
enlargement  from  about  the  tenth  to  the  twelfth  dorsal  vertebrae.  Both 
appear  in  fcetal  life  synchronously  with  the  development  of  the  extremities. 

In  the  spinal  cord  the  gray  substance  is  everywhere  surrounded  by  white 
substance.  Only  the  tips  of  the  posterior  horns  touch  the  periphery  at  the 
place  of  entry  of  the  posterior  roots.  The  changing  formation  of  the  white 
and  the  gray  substance  decides  the  different  forms  seen  in  transverse  section 
of  the  spinal  cord  in  the  various  segments.  Immediately  below  the  pyramidal 
crossing,  the  gray  substance,  by  development  of  the  anterior  and  posterior 
horns,  assumes  the  characteristic  butterfly  figure.  The  anterior  horns, 
the  seat  of  the  great  motor  cells,  are,  naturally,  developed  especially  well  in 
the  cervical  and  lumbar  enlargements.  But  the  development  of  the  gray 
substance  in  the  entire  lumbo-sacral  portion  of  the  cord,  absolutely  as  well 
as  in  relation  to  the  quickly  diminishing  white  substance  is  a  particularly 
strong  one.  In  the  dorsal  portion  of  the  cord  a  process,  which  may  also  be 
observed  between  the  anterior  and  posterior  horn  at  other  points  of  the  cord, 
the  reticular  process  is  developed  into  a  lateral  horn.  The  posterior  horn 
shows  considerable  thickening  where  it  passes  into  the  medulla  oblongata, 
as  well  as  in  the  lower  segments  of  the  sacral  portion  of  the  cord.  At  its 
base  lies  Clarke's  column,  developed  especially  well  in  the  dorsal  portion  of 
the  cord;  the  head  of  the  posterior  horn  follows,  then  the  peculiarly  trans- 
parent gelatinous  substance  of  Rolando,  the  marginal  zone  and  the  apex 
of  the  posterior  horn,  which  is  to  be  regarded  as  belonging  to  the  glial 
covering  of  the  cord.  Both  symmetrical  halves  of  the  gray  substance  are 
bound  together  by  an  anterior  and  a  posterior  gray  commissure,  which 
embrace,  both  in  the  front  and  the  rear,  the  central  canal  with  its  surrounding 
central  gelatinous  substance.  In  front  of  the  anterior  gray  commissure  lies  a 
broad  bridge  of  white  substance,  the  anterior  white  commissure;  but  number- 
less medullated  fibres  pass  also  through  the  gray  commissure. 

The  white  substance  of  the  spinal  cord  is  divided  into  posterior,  lateral, 
and  anterior  columns.  Whereas  the  separation  between  the  posterior  and 
lateral  columns  is  rather  clearly  indicated  by  the  apex  of  the  posterior  horn 
and  the  entering  bundles  of  the  posterior  roots,  no  definite  anatomic  bound- 
ary can  be  established  between  the  lateral  and  anterior  columns;  the  ventral 
border  of  the  roots  which  is,  however,  frequently  interrupted  may  be  con- 
sidered as  the  boundary.  Between  the  two  anterior  columns  lies  the  deep 
anterior  fissure,  which  reaches  the  anterior  white  commissure  that  connects 
the  anterior  columns.  Between  the  two  posterior  columns  is  the  septum 
posterius,  usually  not  quite  opened  into  a  fissure;  it  reaches  the  posterior 
commissure.  Between  the  septum  posterius  and  the  dorsal  line  of  roots, 
which  deepens  into  the  sulcus  lateralis  posterior,  there  is  mostly  a  sulcus 


ANATOMY  OF  THE  SPINAL  CORD  193 

intermedius  posterior,  which  permits  us  to  divide  the  posterior  column  into 
a  lateral  part,  the  column  of  Burdach,  and  a  medial  part,  the  column  of  Goll. 

The  spinal  cord  roots  are  divided  into  anterior  and  posterior.  The 
anterior  roots  which  spring  from  the  anterior  horn  cells  of  the  spinal  cord, 
pass,  separated  into  several  fibres,  through  the  antero-lateral  column  to 
the  periphery,  where  they  unite  into  a  compact  bundle.  The  posterior 
roots  spring  from  the  spinal  ganglia,  which  are  situated  in  the  intervertebral 
foramina,  outside  of  the  dural  sac.  Only  the  lowest  spinal  ganglia,  and, 
exceptionally,  those  of  the  uppermost  cervical  roots,  lie  intradurally  in  the 
vertebral  canal.  Hence  the  posterior  roots  pass  to  the  sulcus  lateralis 
posterior,  in  order  to  penetrate  the  so-called  entry  zones  of  the  roots  of  the 
posterior  column.  While  the  anterior  roots  are  thus  placed  ventrally  to  the 
spinal  ganglia,  the  anterior  and  posterior  roots  unite  laterally  from  the  gang- 
lion into  a  bundle,  the  peripheral  nerve. 

Concerning  the  distribution  of  the  blood  in  the  spinal  cord,  every  vertebral 
artery,  before  union  with  the  basilar  artery,  sends  a  branch  backwards,  and, 
higher  up,  one  to  the  front.  Both  anterior  arteries,  A.  vertebrospinales 
anteriores,  unite,  running  downwards,  in  the  top  part  of  the  spinal  cord, 
into  an  azygos  artery,  which,  in  the  cervical  enlargement,  terminates  in  the 
tractus  arteriosus  spinalis  anterior.  The  latter  is  formed  of  an  anastomotic 
chain  of  the  anterior  short  branches  of  the  intercostal,  lumbral  and  sacral 
arteries,  down  to  the  filum  terminale.  From  this,  the  central  arteries  pass 
through  the  anterior  fissure  to  the  anterior  commissure  and  send  each  a 
branch  into  the  gray  substance  of  either  side;  only  the  peripheral  divisions 
of  the  latter  are  also  supplied  with  blood  from  other  sources.  Furthermore, 
from  the  tractus  arteriosus  spinalis  anterior,  lateral  branches  reach  the 
anterior  roots,  the  anterior  and  antero-lateral  column  region. 

The  posterior  vertebro-spinal  artery  unites  with  the  posterior  anasto- 
motic chain  of  the  intercostal,  lumbar  and  sacral  arteries  to  form  the  trac- 
tus arteriosus  posterolateralis,  one  on  each  side.  From  its  medially  passing 
rami  penetrantes,  a  second  tractus  arteriosus  posterior  is  formed  medially 
from  the  posterior  roots.  From  all  these  vessels,  some  arteries  pass  into 
the  white  substance,  the  vasocorona,  and  supply  this  as  well  as  the  per- 
ipheral parts  of  the  gray  substance.  All  arteries  entering  the  spinal  cord 
are  terminal  arteries. 

The  veins  of  the  spinal  cord,  upon  whose  course  we  can  not  enter  here 
with  greater  minuteness,  are  more  developed  than  the  arteries;  they  are  all 
characterized  by  the  absence  of  valves.  Though  we  know  very  little 
of  the  course  of  the  lymphatic  vessels,  their  product,  the  cerebrospinal 
fluid,  has,  in  recent  years,  attracted  attention  in  a  constantly  increasing 
degree.  This  clear  fluid  of  about  1003  specific  gravity  fills  the  subarach- 
noid space  as  well  as  the  cavities  of  the  central  nervous  system;  it  shows  a 
slightly  alkaline  reaction,  contains  small  quantities  of  globulin,  and  about 
13 


i94  DISEASES  OF  THE  SPINAL  CORD 

i  %  of  solid  substance.  Quincke's  lumbar  puncture,  the  cocainizing  of  the 
spinal  cord,  introduced  by  Bier,  and  in  most  recent  times,  the  serodiagnostic 
syphilis  reaction  of  Wassermann-Neisser-Bruck  have  more  and  more  clearly 
shown  the  importance  of  this  fluid,  which  continually  moistens  the  central 
nervous  system. 

So  far  as  the  microscopically  recognizable  structure  of  the  spinal  cord  is 
concerned,  it  consists,  like  that  of  the  entire  central  nervous  system,  of 
ganglion  cells,  nerve  fibres  and  neuroglia.  Among  the  ganglion  cells  the  large 
cells  of  the  anterior  horn  (anterior  root  cells)  appear  as  the  type  of  the  motor 
ganglion  cell.  They  are  multipolar  and  have  an  axis  cylinder  process, 
and  possess  a  centrally  situated  nucleus  with  nucleoli.  The  axis  cylinder 
process  becomes  continuous  with  a  medullated  nerve  fibre,  which  passes 
ventrad  into  the  anterior  column.  In  the  cell  protoplasm  are  found  NissVs 
granules  (tigroid  bodies),  among  which  an  achromatic  substance  is  found, 
the  main  components  of  which  are  the  neurofibrils;  these  traverse  the  body 
of  the  cell,  and  appear  in  all  processes,  even  in  the  axis  cylinder.  The 
neurofibrils  probably  represent  that  part  of  the  body  of  the  cell,  which  con- 
ducts the  stimulation,  though  nothing  has  been  definitely  ascertained  as  to 
the  relations  of  the  ganglion  cells  to  the  extracellular  fibril  structures.  One 
thing,  however,  seems  to  be  proved  by  the  most  recent  investigations  into  the 
coursing  of  the  fibril,  namely,  that  the  theory  of  the  neuron — that  is,  the  entity 
of  the  ganglion  cell  with  its  axis  cylinder,  which  is  said  to  have  no  connection 
with  other  neurons  except  by  contact,  a  theory  which  has  been  formulated 
under  the  influence  of  Golgi's  methods  of  metallic  impregnation  of  the  cell — 
is  not  tenable  from  the  purely  anatomic  point  of  view.  On  the  other  hand,  we 
must  consider  the  neuron  theory  for  the  present  as  especially  valuable  for 
physiological  and  clinical  investigation,  and  adhere  to  it  because  of  its  great 
importance  for  further  researches. 

In  the  ganglion  cells,  especially  of  the  anterior  horns,  there  is  found  also  a 
peculiar  yellow  pigment,  which  in  adults,  as  a  rule,  is  found  collected  in  clusters 
on  one  side  of  the  protoplasm.  This  pigment,  described  as  lipochrome 
because  of  its  behavior  to  osmium  and  the  fatty  extractive  substances,  is 
entirely  lacking  in  the  newly  born.  It  appears  in  the  child  at  the  age  of  from 
6  to  8,  and  increases  in  quantity  with  advancing  years,  until  in  old  people  of 
from  8o  to  90  it  often  occupies  almost  the  entire  protoplasm  of  the  cell. 
Since  this  phenomenon  appears  with  increasing  years  also  in  animals  (dogs, 
horses,  monkeys)  it  might  be  regarded  as  a  product  of  age,  which  inhibits 
the  activity  of  the  ganglion  cells. 

The  anterior  horn  cells  are  found  at  the  various  levels  of  the  spinal  cord, 
in  varying  numbers,  sizes  and  arrangements,  but  as  a  general  rule,  four 
groups  of  ganglion  cells  may  be  distinguished  by  cross-section,  a  ventro- 
medial and  a  dorsomedial,  a  ventrolateral  and  a  dorsolateral.  The  latter 
two  are  very  strongly  developed,  especially  in  the  cervical  and  lumbo-sacral 


ANATOMY  OF  THE  SPINAL  CORD  195 

portions  of  the  cord.  Of  other  groups  of  cells,  those  in  the  lateral  horn  and 
in  the  intermediate  zone  between  the  anterior  and  the  posterior  horns  are  to 
be  mentioned — the  sympathetic  nuclei.  According  to  the  most  recent 
investigations,  there  are  three  such  nuclear  columns,  the  nucleus  sympathicus 
lateralis  superior  from  the  eighth  cervical  to  the  third  lumbar  segment,  the 
nucleus  sympathicus  lateralis  inferior  from  the  second  sacral  segment  to  the 
coccygeal  portion  of  the  canal  and  the  nucleus  sympathicus  medialis  inferior, 
from  the  fourth  lumbar  segment  to  the  coccygeal  portion  of  the  canal. 

Besides  these,  Clarke's  columns  stand  out  preeminently.  This  group  of 
cells,  situated  at  the  base  of  the  posterior  horn,  dorsolateral  from  the  central 
canal,  is  most  strongly  developed  in  the  dorsal  and  lower  cervical  portions 
of  the  cord,  but  is  well  marked  also  in  all  the  other  portions  of  the  cord. 
Clarke's  columns  are  characterized  by  rather  large  ganglion  cells  with  large 
nuclei  and  nucleoli,  relatively  few  and  coarse-grained  tigroid  bodies,  and 
strikingly  numerous  protoplasmic  processes.  We  cannot  here  enter  into 
the  structure  of  the  rest  of  the  ganglion  cells,  which  in  great  variety  are 
distributed  in  the  spinal  cord. 

The  gray  matter,  as  it  appears  especially  in  Weigert's  hasmotoxylin 
preparations,  is  rilled  by  great  masses  of  coarse  and  fine  medullary  sheath 
nerve  fibres.  Among  them  we  find  a  relatively  small  number  of  fibres 
running  from  above  downwards;  they  run  for  but  a  short  distance.  With 
the  exception  of  the  accessorius  roots  in  the  upper  portion  of  the  cervical 
segment,  which  run  through  the  lateral  column,  only  the  anterior  root  fibres 
leave  the  spinal  cord  from  the  gray  matter.  Besides  these,  there  arise  in 
the  gray  matter  of  the  spinal  cord  fibres  which  pass  up  and  down;  they 
course  in  the  lateral  and  anterior  columns,  and  also,  but  fewer  in  number, 
in  the  posterior  column.  Into  the  gray  matter  pass  fibres  from  the  various 
columns  of  the  white  matter  and  fibres  of  the  posterior  roots;  they  radiate, 
in  part  directly,  and  partly  as  collaterals  of  the  posterior  column  fibres, 
into  the  posterior  horn  and  Clarke's  column,  but,  a  portion  of  them  also  may 
be  traced  to  the  region  of  the  anterior  horn  cells.  Besides  these  there  are 
numberless  commissural  fibres,  which,  passing  through  the  gray  commis- 
sures and  especially  also  through  the  anterior  white  commissure,  connect 
the  two  halves  of  the  gray  matter;  they  also  make  possible  a  connection  of 
the  white  spinal  cord  tracts  with  the  gray  matter  of  the  other  half  of  the 
spinal  cord. 

Finally  relative  to  the  neuroglia,  which  shares  with  the  ganglion  cells  an 
ectodermal  origin,  one  must  distinguish  the  ependymal  cells,  lining  the 
cavities  of  the  central  nervous  system  and  therefore  also  the  central  canal, 
and  the  neuroglia  cells  distributed  throughout  the  gray  and  white  matter, 
which,  however,  seem  to  have  the  same  embryonic  origin.  Intermingled 
with  the  neuroglia  cells,  which  we  must  be  careful  not  to  confuse  with  gang- 
lion cells,  especially  in  pathological  relations,  there  exists  an  extraordinary 


196  DISEASES  OF  THE  SPINAL  CORD 

fine  meshwork  of  glia  fibres.  These  glia  fibres,  however,  are  not  to  be 
regarded  as  independent  formations,  but  as  processes  of  the  glia  cells.  In 
every  loss  of  the  actual  nerve  substance  in  the  spinal  cord,  there  is  a  pro- 
liferation of  the  glious  elements  which  leads  to  the  formation  of  sclerosis. 

If  we  now  consider  the  white  matter  of  the  spinal  cord,  it  consists,  besides 
the  supporting  substances,  exclusively  of  nerve  fibres,  which  serve  to  carry 
intraspinal  and  cerebro-spinal  impulses.  The  separate  bundles  of  nerve 
fibres  bound  together  for  the  conduction  of  special  functions,  are  therefore 
called  conducting  paths.  Since  observation  of  the  normal  sections  of  the 
spinal  cord  could  give  but  little  definite  knowledge  as  to  the  course  of  such 
tracts,  the  secondary  degenerations,  which  appear  in  consequence  of  an  inter- 
ruption of  certain  tracts  in  focal  affections  of  the  central  nervous  system, 
were  early  used  to  investigate  this  matter.  After  a  medullated  nerve  fibre 
is  completely  divided,  there  appears  in  the  peripheral  part,  separated  from 
the  parent  cell,  swelling,  degeneration  and  finally  absorption  of  the  medullary 
sheath.  The  fresh  degeneration  of  medullary  sheaths  may  be  proved  with 
great  accuracy  by  means  of  Marchi's  method,  in  which  osmic  acid  stains 
newly  degenerating  medulla  black;  the  absence  of  medullary  sheaths  in 
older  processes  can  be  readily  demonstrated  by  Weigert's  medullary  sheath 
method.  Only  in  later  stages  of  the  processes,  and  under  peculiar  circum- 
stances, is  there,  besides  this  cellulifugal  degeneration  of  the  peripheral 
nerve  stump,  also  a  retrograde  degeneration  of  the  central  nerve  stump  in 
addition  to  characteristic  changes  in  the  nerve  cells  themselves. 

Another  successful  means  of  acquiring  knowledge  as  to  the  individual 
conducting  tracts  of  the  spinal  cord,  is  Flechsig's  method  for  the  study  of  the 
development  of  the  medullary  sheath,  which  is  based  upon  the  disparity  in 
time  of  the  medullary  development  of  the  individual  tracts.  Then  comes 
Gudden,s  method  of  developmental  inhibition,  which  rests  upon  the  fact  that 
after  destruction  of  a  center  or  a  conducting  path  in  a  newly  born  animal, 
i.  e.,  in  incomplete  development,  all  the  centers  and  tracts  that  belong  to  the 
same  system  fail  of  complete  development. 

Finally  the  experimental  and  physiological  methods  of  irritation  and 
destruction  of  certain  centers  or  conducting  tracts  in  the  grown  animal  are 
important  for  the  establishment  of  the  conducting  tracts. 

Of  the  conducting  tracts  of  the  spinal  cord,  the  longest  known  is  the 
pyramidal  tract  (cortico-spinal  tract).  It  has  its  origin  in  the  region  of  the 
central  convolution  of  the  cortex  of  the  cerebrum,  and,  according  to  the  most 
recent  investigations,  predominantly  in  the  anterior  central  convolution. 
But  the  region  of  its  origin  must  in  any  case  extend  over  the  area  of  the  Betz 
giant  pyramid  cells.  After  its  known  course  through  the  internal  capsule, 
the  basis  of  the  cerebral  peduncle,  and  the  pons,  it  reaches  as  the  pyramid, 
the  ventral  surface  of  the  medulla  oblongata,  and,  where  this  makes  its 
transition  into  the  spinal  cord,  enters  the  pyramidal  crossing,  in  such  a  way 


THE  PYRAMIDAL  TRACTS  197 

that  the  fibres  from  every  pyramid  pass  backwards  and  cross  one  another  at 
an  acute  angle,  in  the  form  of  bundles,  like  the  fingers  of  folded  hands,  ven- 
tral from  the  central  canal.     From  the  crossing  the  pyramidal  fibres  pass 


Fig.  45,  a. — Degeneration  of  both  pyramidal  tracts  of  the  lateral  columns  in? the  first  cervical 
segment,  directly  below  the  destroyed  pyramidal  crossing  in  the  macacus  rhesus.  Descending 
degeneration  in  the  medial  parts  of  the  anterior  columns.     Marchi  preparation. 


Fig.  45,  b. — The  same  degeneration  in  the  seventh  cervical  segment. 

to  the  base  of  the  posterior  horn,  and  then,  with  a  sharp  bend  outwards,  into 
the  lateral  column.  While  this  crossing  in  the  dog  and  even  in  the  lower 
monkeys  is  complete,  so  that  the  pyramidal  tract  connects  each  hemisphere 
of  the  cerebrum  directly  with  the  opposite  lateral  column,  there  is,  in  the 


198 


DISEASES  OF  THE  SPINAL  CORD 


anthropoid  ape  and  especially  in  man,  a  considerable  part  of  the  pyramidal 
tract,  that  takes  no  part  in  the  crossing,  but  passes  uncrossed  downwards 
along  the  anterior  fissure  into  the  anterior  column.  The  relation  of  the 
crossed  part  of  the  pyramidal  tract  (lateral  pyramidal  tract)  to  the  uncrossed 
(anterior  pyramidal  tract)  is  very  changeable  in  man,  so  that  cases  in  which 
the  anterior  tract  is  almost  lacking  are  found,  as  well  as  isolated  cases,  in 
which  the  pyramidal  crossing  is  utterly  lacking  and  the  entire  pyramidal 
tract  reaches  the  anterior  column  uncrossed.  Besides  these  two  kinds  of 
fibres,  there  are  a  small  number  of  pyramidal  fibres,  which,  uncrossed,  reach 
the  lateral  column  on  the  same  side;  these  appear  to  be  somewhat  more 
numerous  in  man  than  in  monkeys  and  dogs,  in  which  they  may  be  occa- 
sionally altogether  absent  (Fig.  45,  a  and  b). 

The  great  variability  of  the  pyramidal  tract  in  man  coincides  with  the 
ever  changing  situation  and  size  of  this  tract  in  animals,  and  points  to  the 


Fig.  46. — Pyramidal  tract  and  rubrospinal  bundle  in  the  spinal  cord  of  a  dog.     a,  Cervical 
enlargement;  b,  dorsal  region.    Py,  pyramidal   tract.    R,  rubro-spinal  bundle. 

comparative  youthful  age  of  this  tract  in  the  animal  kingdom.  Birds  do 
not  have  a  pyramidal  tract  at  all;  it  is  only  indicated  in  the  upper  part  of 
the  cervical  portion  of  the  spinal  column  in  the  hedgehog,  the  sheep  and 
goat;  in  mice,  rats  and  squirrels  it  does  not  pass  into  the  lateral  column, 
but  into  the  top  of  the  posterior  column,  and  we  can  recognize  in  passing 
from  the  rabbit,  by  way  of  the  cat  and  dog,  to  the  monkey,  a  gradual  growth 
and  increase  of  calibre  in  the  fibres  of  the  tract,  which  in  that  species  is 
situated  exclusively  in  the  lateral  column.  Only  in  anthropoids  does  a 
clearly  defined  anterior  pyramidal  tract  exist. 

The  development  of  the  pyramidal  tract  is  also  relatively  late;  its  axis 
cylinders  begin  to  appear  in  man  in  the  sixth  to  seventh  fcetal  month.  The 
medullary  envelopment  does  not  take  place  until  after  birth.  The  lateral 
pyramidal  tract  lies  in  the  postero-lateral  column;  in  the  cervical  portion 
of  the  cord  it  is  separated  from  the  periphery  by  other  masses  of  fibres;  in 
the  lumbo-sacral  portions,  it  is  adjacent  to  the  periphery.     From  it  fine  col- 


LATERAL  PYRAMIDAL,  RUBROSPINAL,  DEITER'S  TRACTS     199 


Fig.  47. — Pyramidal   tract   and    rubro-spinal 
bundle  in  the  ape.     Third  cervical  segment. 


laterals  pass  to  the  base  of  the  anterior  horns,  though  no  one  has,  as  yet 
succeeded  definitely  in  establishing  the  exact  place  where  these  fibres 
terminate  in  the  gray  matter.  At  any  rate,  they  do  not  seem  to  connect 
directly  with  the  cells  of  the  anterior  horn.  The  lateral  pyramidal  tract 
may  be  traced  downwards  into  the  lowest  part  of  the  sacral  portion  of  the 
cord  by  staining  the  tissues  by  Marchi's  degeneration  method;  it  gradually 
decreases  in  volume  as  it  proceeds  downwards.  The  anterior  pyramidal 
tract,  as  a  rule,  does  not  extend  beyond  the  lowest  part  of  the  dorsal  cord. 

In  about  the  same  area  as  the  lateral  pyramidal  tract,  there  runs  a  second 
tract  descending  from  the  brain  to  the  spinal  cord,  the  rubrospinal  tract 
(Monakow's  bundle) .  Its  main  mass 
springs  from  the  red  nucleus  of  the 
corpora  quadrigemina  region,  crosses 
immediately  after  its  origin  in  Forel's 
tegmental  crossing,  and  reaches, 
after  receiving  some  pontile  fibres, 
the  postero-lateral  column,  in  which 
it  passes  down  to  the  sacral  portion 
of  the  cord;  it  sends  out  compara- 
tively large  collaterals  to  the  gray 
matter  at  the  base  of  the  anterior 
horns  (Figs.  46  and  47). 

This  tract  stands  in  inverse  relation  to  the  pyramidal  tract;  in  the  ab- 
sence of  the  latter  it  is  well  developed,  and  as  the  other  increases  in  the  animal 
kingdom  it  gradually  diminishes.  In  the  dog,  the  pyramidal  tract  is  still 
surpassed  by  it  in  size,  surrounding  it  laterally  and  ventrally;  in  the  monkey 
it  may  be  correctly  designated  as  a  prepyramidal  column,  since  the  bulk  of 
its  fibres  is  being  forced  out  ventrad  by  the  strong  development  of  the  pyra- 
midal tract.  In  man,  it  is  only  a  rudimentary  prepyramidally  situated 
little  bundle  of  fibres.  In  about  the  same  area  there  are  also  some  fibres, 
which  descend  from  Deiter's  nucleus  to  the  spinal  cord. 

In  addition  to  the  anterior  pyramidal  tract,  which  is  situated  alongside 
the  anterior  fissure,  the  anterior  column  also  contains  other  important  tracts 
passing  from  the  brain  to  the  spinal  cord.  The  largest  of  these  has  its 
origin  in  Deiter's  nucleus,  a  large  group  of  cells  lying  in  front  of  the  cere- 
bellum at  the  level  of  the  acusticus-nuclei,  and  reaches,  uncrossed  in  the  main, 
the  anterior  and  antero-lateral  columns  of  the  spinal  cord,  whence,  adja- 
cent to  the  periphery,  it  passes  to  the  lowest  part  of  the  sacral  portion. 
This  vestibulospinal  or  Deiter's  tract  is  connected  by  large  collaterals  with 
the  anterior  horn  of  the  same  side. 

A  second  important  connection  between  the  midbrain  and  the  anterior 
column  is  the  corpora  quadrigemina  anterior  tract;  it  arises  from  the  gray 
matter  of  the  anterior  quadrigeminal  body,  crosses  completely  in  Meynert's 


200 


DISEASES  OF  THE  SPINAL  CORD 


tegmental  crossing,  reaches  the  anterior  column  of  the  spinal  cord  by 
way  of  the  posterior  longitudinal  bundle,  where,  by  a  wealth  of  collaterals, 
it  radiates  into  the  anterior  horn.  This  tract  is  paralleled  by  a  pontile 
anterior  tract,  rising  in  the  gray  matter  of  the  pons;  it  likewise  passes  down- 
wards in  the  peripheral  divisions  of  the  medio-anterior  column  (Fig.  48) . 

In  the  most  lateral  portion  of  the  anterior  column,  and  above  all  in  the 
ventrad  antero-lateral  column  of  the  upper  cervical  portion  of  the  cord,  the 
bundles  of  fibres  course,  which  descend  from  the  respiratory  centers  in  the 
medulla  oblongata  to  the  phrenic  centers  in  the  fourth  cervical  segment, 
but  their  course  is  not  exactly  known. 


^ 

E3 

EZ3 

esa 

POQMCDOSI 

I 

2 

3 

4 

5 

Fig.  48. — Descending  cerebro-spinal  anterior  column  tracts  in  the  dog.  1,  Tract  from  Deiter's 
nucleus;  2,  fibres  from  the  pons  to  the  anterior  column  on  the  same  side;  3,  fibres  from  the  pons 
to  the  anterior  column  of  the  opposite  side;  4,  tract  from  the  quadrigeminal  bodies  to  the  anterior 
column ;  5,  fibres  of  the  posterior  longitudinal  bundle. 

In  addition  to  all  the  tracts  here  described,  which  pass  from  the  brain 
into  the  spinal  cord  and  degenerate  descendingly  after  they  are  interrupted, 
and  which  occupy,  as  a  whole,  peripheral  divisions  of  the  spinal  cord,  there 
are  in  all  parts  of  the  anterior  and  lateral  columns  other  intra-spinal  connec- 
tion fibres,  which  pass  through  the  white  matter,  for  shorter  or  longer  dis- 
tances, in  order  to  connect  higher  with  lower  spinal  segments.  The  shorter 
of  these  pass  on  the  very  margin  of  the  gray  matter,  while  the  longer  ones 
approach  the  periphery  and  partly  commingle  with  the  cerebro-spinal 
fibre  systems. 

Now  as  to  the  ascending  systems  of  fibres,  passing  from  the  spinal  cord 
to  the  brain;  there  is  no  such  system,  which  analogous  to  the  pyramidal  tract, 
ascends  directly  to  the  cerebral  cortex.    From  the  posterior  roots,  springing 


ASCENDING  TRACTS  OF  THE  SPINAL  CORD  201 

from  the  spinal  ganglion  cells,  only  one  tract  passes  in  the  spinal  cord,  with- 
out interruption,  up  to  the  medulla  oblongata:  the  tract  of  the  long  posterior 
column  fibres.  But  only  a  small  part  of  the  posterior  root  fibres  that  pass 
upwards  reaches  the  posterior  column  nuclei  of  the  medulla  oblongata, 
whereas  a  large  part  of  them  partly  in  the  segment  of  the  root  entry,  partly 
in  higher  spinal  segments,  unites  with  the  cells  of  the  posterior  horns,  and 
Clarke's  columns.  As  the  bundle  of  fibres,  entering  one  segment  of  the 
posterior  column,  places  itself  along  the  medial  margin  of  the  posterior  horn 
and  is  then  pushed  mediad  by  the  posterior  root  fibres  of  the  next  higher 
segment,  there  arises  an  imbricated  structure  of  the  long  posterior  root  fibres 
in  the  posterior  column,  in  such  a  way  that  finally  in  the  upper  cervical  por- 
tion of  the  cord  the  posterior  root  fibres,  coming  from  the  posterior  roots 
of  the  lumbo-sacral  portion  and  almost  the  entire  dorsal  portion,  are  placed 
in  the  medial  part  of  the  posterior  column,  the  so-called  GolVs  column,  and 
end  in  the  medio-posterior  column  nucleus  of  the  medulla  oblongata,  the 
nucleus  funiculi  gracilis,  while  the  laterally  situated  Bur  (lack's  column  is 
supplied  by  the  posterior  root  fibres  of  the  cervical  and  upper  dorsal  portions 
of  the  cord,  and  ends  in  the  nucleus  funiculi  cuneati  of  the  medulla  oblon- 
gata (Fig.  49).  None  of  the  fibres  of  the  posterior  column  goes  higher  than 
this;  only  by  the  help  of  the  lemniscus  tract,  rising  in  the  posterior  column 
nuclei  of  the  medulla  oblongata  and  passing  to  the  optic  thalamus,  and  by 
the  thalamo-cortex  connections  is  an  indirect  connection  with  the  cortex  of 
the  cerebrum  established. 

This  posterior  column  portion  of  the  posterior  roots  passing  directly 
to  the  posterior  column  nuclei  of  the  medulla  oblongata,  increases  gradually 
n  the  ascending  scale  of  animals,  and  is  most  developed  in  man. 

In  addition  to  the  large  ascending  posterior  column  tract,  every  posterior 
root  sends  off,  at  its  entry  into  the  posterior  column  a  small  descending 
branch.  It  consists  of  a  strand  of  fibres,  which  may  be  traced  downwards  a 
few  segments  from  the  root  entry;  at  the  same  time  it  gradually  moves  away 
from  the  posterior  horn,  and  attenuates  very  quickly. 

Besides  these  exogenous  or  root  fibres  of  the  posterior  column,  there  exist 
also  endogenous  fibres,  originating  in  the  gray  matter  of  the  spinal  cord. 
Our  knowledge  of  these  we  owe  partly  to  experimental  destruction  of  the 
gray  matter  in  the  lumbar  portion  of  the  cord,  as  it  may  be  produced  by 
ansemization  in  consequence  of  temporary  occlusion  of  the  aorta  abdom- 
inalis  or  by  embolization  of  the  terminal  arteries  of  the  gray  matter;  partly 
also  to  the  study  of  fresh  cases  of  poliomyelitis,  in  which  only  the  gray  sub- 
stance is  affected  in  the  more  or  less  extended  process  of  inflammation.  In 
such  lesions  of  the  gray  substance  of  the  lumbo-sacral  portion  of  the  cord, 
one  sees  at  the  level  of  the  lesion  the  ventral  top  of  the  posterior  column,  the 
cornu- commissural  zone,  filled  by  moderate  degeneration.  Hence  the  field 
of  degeneration  extends  as  a  dorsal  endogenous  posterior  column  field,  in 


202 


DISEASES  OF  THE  SPINAL  CORD 


an  upward  direction,  dorsad  along  the  posterior  fissure,  and  reaches,  with 
a  small  part  of  its  fibres  mixed  with  the  posterior  root  fibres  of  the  sacral 
portion  of  the  cord,  Goll's  nucleus  of  the  medulla  oblongata  (Fig.  50). 

Descending  degenerating  posterior  column  fibres,  described  as  endoge- 
nous systems,  have  become  known,  more  especially  through  pathological 
observations.  These  form  Schultze's  comma  shaped  tract  at  the  border  of 
Goll's  and  Burdach's  columns  in  the  cervical  and  upper  part  of  the  dorsal 
portion,  Flechsig's  oval  field  at  the  posterior  fissure  in  the  lumbar  portion 
and  the  medio-triangtdar  tract  in  the  dorsomedial  angle  of  the  lower  sacral 
portion  of  the  cord.  These  fields  in  their  course  from  above  downwards 
seem  to  merge  into  one  another,  but  whether  they  are  purely  endogenous 
descending  posterior  column  fibres,  or  are  mixed  with  posterior  root  fibres, 
has  not  been  definitelv  ascertained. 


Fig.  49,  a  and  b. — Degeneration  of  the  posterior  column  after  total'destruction  of  the  fifth 
lumbar  root,  a,  Upper  part  of  the  fifth  lumbar  segment;  b,  twelfth  dorsal  segment.  {After 
Schaffer.) 

Direct  posterior  root  fibres  end  only  in  the  posterior  columns.  The 
connection  of  the  posterior  roots  with  the  ascending  systems  of  lateral  and 
anterior  columns  results  exclusively  through  the  mediation  of  the  ganglion 
cells  of  the  gray  matter.  Most  important  here,  are  the  spino-cerebellar 
tracts  of  the  lateral  column,  which  are  divided  into  a  dorsal  or  Flechsig's 
tract,  and  a  ventral  tract  or  Gowefs  bundle.  The  dorsolateral  cerebellar 
tract  springs  from  the  ganglion  cells  of  Clarke's  column,  in  the  dog  from  the 
sacral  portion  on  throughout  the  entire  spinal  cord,  in  man  only  beginning 
with  the  lumbar  enlargement;  it  lies  laterally  from  the  pyramidal  tract  and 
the  rubro-spinal  bundle,  along  the  periphery  of  the  dorso-lateral  column 
and  extends  to  the  apex  of  the  posterior  horn.  In  its  course  upward,  it 
sends  off  numerous  little  fibres  into  the  gray  matter,  at  points  but  reaches 
the  medulla  oblongata  as  a  compact  bundle,  enters  the  inferior  cerebellar 
peduncle,  and  terminates,  mostly  crossed,  in  the  superior  vermis  of  the 
cerebellum. 

While  this  tract  was  first  demonstrated  by  Flechsig  on  the  basis  of  medul- 


THE  VENTROLATERAL  CEREBELLAR  TRACT 


203 


lary  sheath  development,  the  ventrolateral  cerebellar  tract,  Gower's  bundle, 
owes  its  discovery  to  the  degeneration  method.  Its  origin  in  the  spinal  cord 
is  not  quite  definitely  known;  but  the  ventral  cells  of  the  posterior  horn  of 
both  sides  seem  to  send  fibres  to  it.  It  lies  in  the  spinal  cord  in  the  ventral 
half  of  the  lateral  column  along  the  periphery,  dorsad  directly  touching  the 
dorsolateral  cerebellar  tract,  but  mediad  pressing  farther  into  the  white 
matter  of  the  lateral  column  in  the  form  of  a  triangle.  This  tract,  also,  con- 
stantly receives  fibres  in  its  course  through  the  spinal  cord,  and  sends  other 
fibres  off  to  the  gray  matter.  The  main  bulk  of  these  fibres  diverge  in 
the  medulla  oblongata  from  Flechsig's  tract  where  this  tract  enters  the  in- 
ferior cerebellar  peduncle;  it  describes  a  peculiar  curve  over  the  trigeminal 
root  at  its  exit  and  over  the  superior  peduncle  of  the  cerebellum,  thus 
reaching  the  dorsal  division  of  the  vermis  in  which  it  ends,  partly  crossed 
and  partly  uncrossed. 


Fig.  50. — General  view  of  the  ascending  endogenous  degeneration  of  the  right  posterior  column 
after  cutting  off  the  blood  supply  to  the  gray  matter  of  the  sacral  and  lumbar  cord  through 
lycopodium  embolism  in  the  dog.  1,  Sacral  cord;  2-5,  lumbar  cord;  6-8,  dorsal  cord;  0, 
cervical  cord,  a,  Endogenous  ventral  region  of  the  posterior  column;  b,  endogenous  dorsal 
region  of  the  posterior  column;  c,  tractus  septo-marginalis. 

The  termination  of  part  of  the  fibres  of  Gower's  tract  in  the  corpora 
quadrigemina  region,  without  connection  with  the  cerebellum,  though 
stated  by  many  authors,  is  not  established  with  certainty. 

Besides  these  long  ascending  tracts,  there  course  in  the  lateral  columns 
a  great  number  of  short  fibres;  they  run  upwards  for  a  few  segments,  passing 
along  in  the  remnants  of  the  lateral  column  near  the  gray  matter. 

In  the  anterior  column  also,  throughout  the  entire  spinal  cord,  long  bun- 
dles of  fibres  course  upwards  along  the  anterior  fissure  and  the  ventral  per- 
iphery, to  be  gradually  lost  in  its  uppermost  divisions.     They  come,  in  the 


2o4  DISEASES  OF  THE  SPINAL  CORD 

main,  from  the  gray  matter  of  the  other  side,  from  which  they  reach  the 
anterior  column  through  the  anterior  commissure.  From  the  upper  cervical 
portion  of  the  cord,  however,  one  can  trace  fibres,  which,  arising  in  the 
gray  matter,  reach  the  crossed  anterior  column  by  way  of  the  anterior  com- 
missure, through  the  lateral  division  of  the  medial  fillet  and  up  to  the  optic 
thalamus,  so  that  here  a  second  spino-thalamic  connection  is  present  besides 
the  above  mentioned  one  of  the  long  posterior  tracts.  Still  other  fibres 
from  the  anterior  column  reach  the  region  of  Deiter's  nucleus  by  way  of  the 
reticular  formation  of  the  medulla  oblongata.  Besides  these  long  paths, 
there  are  in  the  anterior  column  a  large  number  of  short  ascending  fibres, 
which  after  a  few  segments  end  again  in  the  gray  matter. 

We  can,  therefore,  group  the  conducting  tracts  of  the  spinal  cord,  as 
follows : 

I.  Descending  tracts. 

(a)  Lateral  column. 

i.  Lateral  pyramidal  tract  (tractus  cortico-spinalis  cruciatus). 

2.  Rubro-spinal  tract  (Monakow's  bundle). 

3.  Fibres  from  Deiter's  nucleus. 

4.  Respiratory  tract  in  the  antero-lateral  column. 

5.  Intraspinal  fibres. 

(b)  Anterior  column. 

1.  Anterior  pyramidal  tract  (tractus  cortico-spinalis  ant.). 

2.  Vestibulo-spinal  tract. 

3.  Corpora  quadrigemina  anterior  tract. 

4.  Anterior  pontile  tract. 

5.  Respiratory  tract. 

6.  Intraspinal  fibres. 

(c)  Posterior  column. 

1.  Descending  posterior  root  fibres. 

2.  Descending  endogenous  fibres   (Schultze's  comma,  Flechsig's 

oval  field,  medio-triangular  tract). 

II.  Ascending  paths. 

(a)  Lateral  column. 

1.  Dorsolateral    cerebellar    tract     (Flechsig)     (tractus    cerebello- 

spinalis  dorsalis). 

2.  Ventrolateral    cerebellar    tract    (Gowers)    (tractus    cerebello- 

spinalis  ventralis) . 

3.  Intraspinal  fibres. 

(b)  Anterior  column. 

1.  Ascending    anterior    column    tract    (faisceau    sulco-marginal 
ascendant  Marie). 


REFLEX  ACTIVITY  OF  THE  SPINAL  CORD  205 

2.  Spinothalamic  tract  from  the  upper  cervical  portion  of  the 

spinal  cord. 

3.  Tract  to  the  region  of  Deiter's  nucleus. 

4.  Intraspinal  fibres. 
(c)  Posterior  column. 

1.  Ascending    posterior   column    tract    to    the    posterior    column 

nuclei    of    the    medulla    oblongata:  GolPs    and    Burdach's 
columns. 

2.  Posterior  root  fibres  to  the  higher  spinal  cord  segments. 

3.  Endogenous  fibres    (cornu-commissural    zone  and  dorsal  en- 

dogenous field). 

Function  of  the  Spinal  Cord 

If  we  now  turn  to  the  function  of  the  spinal  cord,  we  must  first  of  all  keep 
before  our  eyes  the  peculiar  position  of  this  central  organ  which,  with  the 
development  of  the  brain,  has  sacrificed  to  it,  more  and  more,  the  peculiar 
independence  possessed  by  the  cord  in  lower  forms  of  animals  and,  as  a 
whole,  has  become  an  intermediate  organ  between  the  centripetal  stimuli 
coursing  into  the  brain  from  the  periphery  of  the  body  by  way  of  the  pos- 
terior roots,  and  the  impulses  sent  from  the  brain  centers  to  the  peripheral 
organs.  The  former  independent  function  of  the  spinal  cord  finds  expres- 
sion now  only  in  reflex  activity,  and  even  this  has  in  great  measure  come 
under  the  dominion  of  the  brain  centers.  Only  after  the  two  have  been 
completely  separated,  does  a  series  of  reflex  phenomena  appear  in  the 
isolated  spinal  cord,  which  must  be  attributed  to  its  "isolation  changes." 

For  the  production  of  a  reflex,  there  is  needed,  besides  the  afferent  and 
efferent  nerve  paths,  a  connecting  link,  the  reflex  center.  From  the  afferent 
nerve,  the  reflex  center  and  the  efferent  nerve  the  reflex  arc  is  formed,  the 
functional  intactness  of  which  is  necessary  to  the  reflex  action.  For  the 
spinal  cord  the  Magendie-BelVs  law  is  of  fundamental  importance;  accord- 
ing to  this  only  the  anterior  roots  assume  motor  conductivity,  whereas  to 
the  posterior  alone  is  assigned  the  sensory  conductivity,  a  law,  which  in  its 
classic  form,  was  first  demonstrated  in  the  frog  by  J  oh.  Mutter.  Even  if  in 
more  recent  times  some  observations  have  appeared,  according  to  which, 
in  the  frog,  some  vasodilatation  impulses  leave  the  spinal  cord  by  posterior 
roots,  the  general  applicability  of  the  law  for  mammals  has  not,  as  yet, 
been  shaken. 

By  this  law,  the  direction  in  which  the  reflex  processes  have  to  take 
place  in  the  spinal  cord,  is  firmly  established.  There  are  reflexes,  which 
have  their  entire  reflex  arc  in  one  segment  of  the  spinal  cord,  whereas  in 
other  reflexes,  various  segments  of  the  spinal  cord,  indeed  even  cerebral 
centers  are  concerned.     In  the  entire  conception  of  the  reflex  process  it  is 


206  DISEASES  OF  THE  SPINAL  CORD 

unquestionably  a  matter  of  importance,  whether  we  must  assume,  according 
to  the  neuron  theory,  a  leap  of  the  centripetal  stimulus  over  to  the  motor 
ganglion  cells,  or  whether  we  must  assume  that  the  conduction  of  the  stimu- 
lus takes  place  through  a  fibrillary  network  without  spacial  interruption. 
However  that  may  be  we,  at  any  rate,  will  ascribe  to  the  ganglion  cells  the 
main  role  in  these  reflex  transitions,  and  will  not  consider  the  extracellular 
fibrillary  net  as  the  central  place.. 

The  well-known  experiment  of  Bethe  on  the  black-clawed  crab,  in  which 
a  separation  of  the  afferent  and  efferent  fibres  of  an  antenna  from  the  brain 
ganglion  did  not  remove  the  possibility  of  reflex  stimulation  of  the  feeler, 
seems  to  speak  for  the  possibility  of  the  summation  of  the  stimuli  and  the 
tonus  without  ganglion  cells.  But  a  simple  transference  of  these  relations 
to  the  reflex  processes  in  vertebrates  is  not  feasible. 

For  the  production  of  reflexes,  Pfliiger  has  laid  down  a  series  of  conduc- 
tion laws,  which  we  reproduce  in  abbreviated  form  (according  to  Leyden 
and  Goldscheider) : 

i.  The  Law  of  Equilateral  Conduction  for  Unilateral  Reflexes 

Whenever  muscular  movements  strictly  confined  to  one-half  of  the 
body  follow  as  reflexes  upon  a  stimulus,  which  affects  a  peripheral  sensory 
nerve,  these  movements  without  exception  and  under  all  circumstances, 
are  upon  the  same  half  of  the  body  to  which  the  stimulated  sensory  nerve 
belongs. 

2 .  The  Law  of  the  Symmetry  of  Reflexes 

If  the  change  in  the  central  organ,  which  is  produced  by  a  stimulated 
sensory  fibre,  has  already  evoked  unilateral  reflexes  and,  in  its  further 
spread,  motors  of  the  opposite  half  of  the  spinal  cord  are  also  excited,  that  is, 
bilateral  reflexes  are  produced,  always  and  under  all  circumstances  only 
such  motors  are  affected,  as  are  already  excited  on  the  primarily  affected 
side,  so  that  bilateral  reflexes  are  never  produced  in  the  opposite  direction. 

3.  The  Unequal  Development  of  the  Reflexes  on  Both  Sides  of  the  Body  in 

Bilateral  Reflexes 

If  the  irritation  of  a  sensory  fibre  induces  reflexes  in  both  halves  of  the 
body,  and  they  are  more  intensive  and  violent  on  one  side,  than  upon  the 
other,  the  stronger  are  always  upon  that  side,  to  which  the  stimulated 
centripetal  fibre  belongs. 

4.  The  Law  of  Inter  sensitive-motor  Movement  and  Reflex-irradiation 

The  direction  from  the  sensory  to  the  motor  nerve  in  the  central  organ, 
is  turned  in  the  brain,  from  before  backward,  in  the  spinal  cord  from  below 
upward,  that  is  in  both  cases  towards  the  medulla  oblongata. 


THE  PATELLAR  AND  ACHILLES  TENDON  REFLEXES       207 

Even  though  these  laws  of  Pflilger  are  on  the  whole  valid,  there  are 
nevertheless  exceptions.  There  are  found  exclusively  crossed  reflexes; 
even  in  the  spinal  cord  reflexes  may  spread  from  above  downwards,  etc. 

The  movements  executed  by  reflexes,  have,  on  the  whole,  the  character 
of  purposeful  reactions.  As  a  rule  there  is  a  co-operation  of  several  muscles. 
Many  of  these  reflexes  may  be  designated  as  protective  reflexes,  as,  for  in- 
stance, the  decapitated  frog's  withdrawal  of  his  foot  from  diluted  acid,  but 
even  the  reflexes  that  serve  vegetative  life,  which  regulate  the  activity  of  the 
bowels,  the  bladder,  the  act  of  birth,  are  quite  suitable  to  the  purpose. 

Of  the  greatest  importance  for  the  entire  spinal  cord  pathology  was  the 
discovery  of  the  patellar  reflex  in  1875;  this  presents  the  most  typical  example 
of  the  tendon  reflexes.  These  tendon  reflexes  consist  in  the  contraction  of  a 
muscle,  or  of  a  group  of  muscles,  on  striking  the  tendon  of  the  muscle. 
Even  though  Westphal  at  first  would  have  it  that  these  reactions  are  only 
an  expression  of  the  direct  muscular  irritation  evoked  by  the  shock,  the 
view  of  Erb,  that  a  spinal  reflex  is  concerned,  is  the  opinion,  which  at  present 
is  the  generally  accepted  theory.  In  the  patellar  reflex  the  reflex  arc  is  made 
up  of  the  sensory  branches  of  the  crural  nerve,  the  quadriceps  center  in  the 
second  to  the  fourth  lumbar  segment,  the  corresponding  motor  roots  and 
motor  fibres  of  the  crural  nerve.  An  injury  in  the  realm  of  any  of  these 
divisions,  and  even  in  the  quadriceps  muscle  itself,  can  abolish  the  patellar 
reflex,  even  though  the  reflex  arc  is  not  completely  destroyed.  This  is 
proven  by  the  return  of  the  reflex  in  a  cerebral  disease  which  increases  the 
excitability  of  the  anterior  horn  cells  even  though  it  has  been  lost  for  a 
considerable  period  of  time  (hemiplegia  in  tabetics).  In  affections  of  the 
cerebro-spinal  descending  tracts,  especially  of  the  pyramidal  tract,  exaggera- 
tion of  the  patellar  reflex  to  a  high  degree  is  always  to  be  found.  Even  by 
irritation  of  the  reflex  center  itself,  such  as  is  evoked  by  strychnine  and 
tetanus  poison,  such  a  reflex  exaggeration  may  be  caused.  In  animals  the 
complete  separation  of  the  spinal  cord  from  the  higher  centers  leads  after 
a  brief  stage,  during  which  the  reflex  is  diminished,  to  a  considerable  in- 
crease of  it.  On  the  other  hand,  a  series  of  pathological  observations  in 
man,  in  contrast  to  the  animal  experiments,  seemed  to  prove  a  total  loss  of 
the  tendon  reflexes  after  complete  transverse  section  of  the  spinal  cord. 
But  since  in  most  recent  times  a  series  of  trustworthy  observations  of  the 
retention  or  even  increase  of  the  tendon  reflexes  with  a  totally  divided  spinal 
cord  have  been  made,  peculiar  complications  must  be  involved  in  those 
cases,  in  which  the  reflex  is  absent.  Probably,  in  these  cases,  abnormal 
changes  exist  in  the  realm  of  the  reflex  arc  itself. 

Completely  analogous  to  the  patellar  reflex  is  the  Achilles  tendon  reflex,  the 
center  of  which  is  situated  in  the  upper  sacral  portion  of  the  cord,  and  the 
triceps-reflex  in  the  arm,  the  reflex  center  of  which  corresponds  to  the  sixth 
to  seventh  cervical  segment.     In  injury  to  the  cerebro-spinal  motor  conduct- 


208  DISEASES  OF  THE  SPINAL  CORD 

ing  tracts,  there  occurs  not  only  an  increase  of  the  tendon  reflexes,  but 
there  appear  immediately  in  the  legs  peculiar  clonic  phenomena,  the  ankle 
clonus  and  the  patellar  clonus,  which  are  induced  by  stimulation  of  the  sen- 
sory nerves  of  the  tendons  and  muscles,  the  tendons  being  in  a  constant 
state  of  tension. 

In  addition  to  the  tendon  reflexes,  the  skin  reflexes  are  of  great  impor- 
tance. In  man  the  plantar,  the  abdominal  and  the  cremaster  reflex  have 
attained  clinical  importance  above  all  others.  The  reflex  arc  of  the  skin 
reflexes  is  not  limited  to  the  spinal  cord,  but  reaches  up  to  the  brain,  partly 
even  up  to  the  cortex  of  the  cerebrum.  The  tactile  reflex  of  the  toes  in  the 
dog,  which  is  elicited  by  touching  the  back  of  the  foot,  and  is  analogous  to 
the  human  skin  reflexes,  is  permanently  lost  after  the  removal  of  the  center 
for  the  extremities  in  the  cerebral  cortex.  In  hemiplegia,  the  skin  reflexes 
on  the  side  of  the  paralysis  are,  at  least  temporarily,  abolished. 

Study  of  the  tactile  reflex  of  the  dog  in  cases  of  disjunction  of  cerebro- 
spinal tracts,  has,  as  a  matter  of  fact,  proven  that  the  construction  of  the 
reflex  arc  for  the  skin  reflexes  is  very  complicated.  Not  only  the  afferent 
limb,  but  also  the  efferent  limb  of  the  tactile  reflex  possesses  two  different 
conducting  tracts  (ascending  posterior  and  anterior  column  tracts,  descend- 
ing pyramidal  tract  and  rubro-spinal  bundle),  so  that  it  can  be  made  to  dis- 
appear only  after  the  destruction  of  either  both  afferent  or  both  efferent 
tracts. 

In  most  recent  times,  the  Babinski  toe  reflex  has  finally  proven,  that  at 
least  one  of  the  skin  reflexes  possesses,  under  pathological  conditions,  besides 
the  cortical  reflex  arc,  a  subcortical  one.  The  toes  show  under  normal 
circumstances  a  distinct  plantar  flexion  upon  irritation  of  the  sole  of  the 
foot;  but  in  injuries  of  the  brain  or  spinal  cord,  which,  as  a  rule,  lead  to  an 
increase  of  the  tendon  reflexes  and  to  spasticity,  a  dorsal  flexion  of  the  toes 
appears.  This  phenomenon  is  the  normal  reflex  process  in  children  during 
the  first  year  of  life,  and  is  only  supplanted  by  plantar  flexion,  probably 
connected  with  the  assumption  of  erect  walking  by  man.  Correspondingly, 
in  the  lower  apes,  such  plantar  flexion  of  the  toes  is  lacking  under  normal 
conditions. 

The  plantar  reflexes  have  their  portals  of  entrance  and  exit  in  the  first 
two  sacral  roots.  The  cremaster  reflex,  which  consists  in  raising  the  tes- 
ticles by  contraction  of  the  cremaster  muscle,  when  the  inner  surface  of  the 
thigh  is  irritated,  is  carried  by  the  first  and  second  lumbar  roots.  The 
abdominal  reflexes,  which  are  not  always  constant,  are  divided  into  an 
upper,  middle  and  lower;  they  are  connected  with  the  seventh  to  the 
eleventh  dorsal  roots. 

Besides  these  skin  and  tendon  reflexes,  the  spinal  centers,  that  are  con- 
nected with  the  sympathetic  nervous  system,  govern  a  series  of  functions. 
The  fibres  of  the  spinal  cord  that  pass  to  the  sympathetic,  begin  only  in 


REFLEX  CENTERS  IN  THE  SPINAL  CORD 


209 


Mid-brain. 


Cervical  swell- 
ing. 


Dorsal  I  to 
lumbar  II  or 
III. 


definite  divisions,  that  is,  from  the  first  dorsal,  occasionally,  too,  from  the 
eighth  cervical  segment  to  the  second  or  third  lumbar  segment,  and  from  the 
second  to  the  fourth  sacral  segment.  The  sympathetic  fibres  pass  out  from 
the  spinal  cord  with  the  anterior  roots,  and  reach  the 
sympathetic  ganglia  (Fig.  51). 

At  the  level  of  the  first  dorsal  segment  lies  the  cilio- 
spinal  center,  the  irritation  of  which  causes  dilatation;  its 
loss,  contraction  of  the  pupil.  It  is  subject  to  the  influence 
of  a  higher  center  in  the  medulla  oblongata,  so  that  a  uni- 
lateral total  division  of  the  cervical  spinal  cord  induces  like- 
wise miosis  of  the  pupil  on  the  same  side;  this,  however,  is 
transitory. 

The  sympathetic  fibres  from  the  dorsal  and  lumbar 
portions  of  the  cord  are  of  marked  importance  for  the  in- 
nervation of  the  vessels.  Vasoconstrictors  and  vasodilators 
for  the  vessels  of  the  skin,  muscles,  intestines,  seem  to 
be  influenced  by  the  spinal  cord.  Especially  important 
are  the  centers,  which,  in  the  upper  sacral  portion,  in- 
fluence the  bladder,  rectum,  and  the  genital  apparatus. 
Experiments  in  recent  years  have  proved,  that  even  after 
disjunction  of  the  gray  substance  of  the  lumbo-sacral  por- 
tion, or  after  removal  of  the  entire  lower  divisions  of  the 
spinal  cord,  the  evacuation  of  the  bowels  and  bladder  is 
still  regulated  after  a  fashion,  so  that  under  pathological 
conditions,  centers  independent  of  the  spinal  cord  step  into 
active  work;  but  it  is  certain,  that  the  normally  regulated 
function  of  bladder  and  rectum  is  under  the  control  of 
the  spinal  cord;  even  where  a  total  division  of  the  cord  oc- 
curs at  a  high  level,  there  appears  again,  after  a  time,  in- 
voluntary evacuation  of  the  bladder,  but  at  regular  inter- 
vals. The  centers  for  the  detrusor  and  sphincter  of  the 
bladder  and  for  the  unstriped  muscles  of  the  rectum,  lie  in 
the  third  to  fifth  sacral  segment,  for  the  external  sphincter 
ani  in  the  fourth  and  fifth  sacral  segment,  for  the  anal 
reflex  in  the  fifth  sacral  segment. 

In  the  upper  sacral  portion  of  the  cord,  there  are  also 
the   centers  for  erection  and  ejaculation.     But  here  too  the  yous  system  in  man. 

.  ,.  {After  Langley.) 

sympathetic  ganglia,  irrespective  of  the  spinal-cord  centers, 
seem  to  possess  a  certain  independence  of  their  own.     Far  more  extensive 
is   the  same  for  the  female  genital  apparatus;  in  animals  with  shortened 
spinal  cords,  as  well  as  in  human  beings,  after  destruction  of  the  lowest 
portion  of  the  spinal  cord,  normal  births  have  been  observed. 

The  independent  function  of  the  spinal    cord   strikes   us   most   clearly 
14 


Sacral  II-IV. 


Fig.    51. 
autonomous 


-The 
ner- 


2IO 


DISEASES  OF  THE  SPINAL  CORD 


when  it  is  separated  from  the  higher  centers.     In  the  decapitated  frog,  one 
can  observe  a  great  series  of  purposeful  movements;  thus,  when  one  of  its 


Fig.  52,  a. — Scheme  of  cutaneous  sensibility  corresponding  to  the  distribution  of  the 
posterior  spinal  roots.     (After  Seiffer.) 

legs  is  held  fast,  it  makes  attempts  to  extricate  it  with  the  other,  reacts  with 
perfect  appropriateness  to  abnormal  skin  stimulations,  etc.     But  even  in 


REFLEX  CENTERS  IN  THE  SPINAL  CORD 


211 


the  dog,  peculiar  reflexes  appear  after  isolation  of  the  spinal  cord,  as  theiwell- 
srudied  scratch  reflex,  the  rhythmical  pawing  of  the  hind  legs,  when  the 


Fig.  52,  b. — Scheme  of  cutaneous  sensibility  corresponding  to  the  distribution  of   the 
posterior  spinal  roots.     (After  Seiffer) 

animal  is  lifted  high  up  (Freusberg's  phenomenon).     Indeed,  with  forward 
motions  of  the  front  part  of  the  body,  which  is  connected  with  the  brain, 


2i2  DISEASES  OF  THE  SPINAL  CORD 

there  may  be  a  lifting  and  a  certain  running-like  movement  of  the  hind 
legs,  as  the  result  of  the  reflex  activity  of  the  isolated  spinal  cord.  In  man, 
of  course,  such  independence  of  the  isolated  spinal  cord  no  longer  exists. 
But  here,  too,  there  may  be  a  series  of  abnormal  reflexes  and  muscle 
twitchings. 

While  in  many  invertebrates  the  entire  body  is  divided  into  numerous 
segments,  to  which  a  similarly  divided  central  organ  corresponds,  this 
division  in  vertebrates  is  demonstrable  only  during  embryonal  life.  In 
later  life  this  segmentation  is  externally  recognizable  only  in  the  vertebral 
column  and  in  the  origin  of  the  spinal  roots.  In  spite  of  this,  every  spinal- 
cord  segment,  characterized  by  the  entrance  and  exit  of  definite  roots,  is  in 
relation  with  definite  parts  of  the  surface  of  the  body  and  definite  skeletal 
muscles.  Thus  one  may  speak  of  a  segment  innervation  of  the  skin,  as  every 
posterior  root  supplies  a  connected  skin  region.  Animal  experiments 
have  taught  us  that  the  realms  of  the  single  posterior  roots  overlap,  so  that 
in  order  to  produce  total  anaesthesia  in  a  portion  of  skin,  not  only  the  main 
root  but  also  the  one  above  and  below  must  always  be  destroyed.  In  addi- 
tion to  the  schemes  offered  by  these  experimental  investigations,  schemes  of 
the  radicular  innervation  of  the  skin  have  been  outlined  relatively  exact  by 
the  results  of  pathologic  studies;  first  of  all,  by  the  phenomena  of  loss  of 
function  observed  in  tumors  and  compressions  of  the  spinal  cord,  as  well 
as  by  the  peculiar  trophic  disturbances,  corresponding  to  the  sensory  zones 
in  herpes  zoster,  a  disease  based  upon  inflammation  of  individual  spinal 
ganglia.  In  recent  times  they  have  come  to  be  of  the  greatest  practical  im- 
portance for  the  exact  localization  of  the  height  at  which  spinal-cord  tumors 
exist  and  without  which  successful  operative  treatment  is  impossible. 

The  sensibility  scheme  (Fig.  52,  a  and  b)  of  Seiffer  reproduced  here 
shows  as  the  most  important  boundaries :  the  parietal-ear-chin  line,  which 
separates  the  trigeminus  area  from  the  cervical  region;  of  next  importance, 
the  neck-trunk  boundary,  anteriorly  it  is  in  the  second  intercostal  space, 
posteriorly  it  corresponds  to  the  spines  of  the  fifth  to  seventh  cervical  vertebras 
where  the  zones  of  the  fourth  cervical  segment  and  the  second  dorsal  seg- 
ment meet.  After  the  fcetal  outgrowth  of  the  front  extremities,  the  skin 
regions  corresponding  with  the  fifth  cervical  to  the  second  dorsal  segments, 
are  distributed  at  the  front  and  back  of  the  arms.  Of  importance  also  are 
the  intermamillary  line  (fourth  to  fifth  dorsal  segment),  the  xiphoid  line 
(sixth  to  seventh  dorsal  segment) ,  and  the  navel  line  (tenth  dorsal  segment) . 
The  trunk-leg  line,  formed  in  front  by  Poupart's  ligament,  behind  by  the 
first  to  second  sacral  vertebra,  separates  the  twelfth  dorsal  and  first  lumbar 
segment.  The  divisions  from  the  second  lumbar  segment  to  the  second 
sacral  segment  serve  for  the  skin  innervation  of  the  legs,  while  the  lowest 
sacral  segments  subserve  the  genital  and  anal  spheres.  Thereby  it  is  note- 
worthy that  a  lesion  of  the  upper  sacral  portion  may  produce  anaesthetic 


SEGMENTARY  DISTRIBUTION  OF  THE  SPINAL  ROOTS      213 

disturbances  at  the  inner  edge  of  the  foot  and  in  the  genital  area,  while  the 
intermediate  skin  regions  remain  undisturbed. 

But  not  only  the  skin  innervation  is  dependent  on  the  segmentary  dis- 
tribution of  the  roots,  but  in  the  anterior  roots  also  there  is  a  peculiar 
segmentary  arrangement  of  the  innervation  of  the  muscles  of  the  body,  which 
swerves  far  from  the  distribution  in  the  peripheral  nerves  and  the  muscles 
themselves.  Thus  every  anterior  root  takes  part  in  the  innervation  of 
several  muscles,  which  may  be  supplied  by  different  peripheral  nerves,  and 
again  a  series  of  anterior  roots  may  take  part  in  the  innervation  of  one 
muscle. 

The  motor  effects  accomplished  by  the  stimulation  of  anterior  roots, 
have  become  known  to  us,  as  a  whole,  by  experiments  on  monkeys.  Con- 
cerning stimulation  of  anterior  roots  in  man,  we  have  only  isolated  information 
at  our  disposal  (see  following  scheme). 

The  Motor  Effects  of  the  Electric  Stimulation  of  the  Anterior  Roots 
in  the  Ape.     {From  Sherrington.) 

Cervicalis. 

I.  Lateral  flexion  of  the  neck  without  rotation  of  the  head. 
II.  Lateral  flexion  and  retraction  of  the  neck,  very  slight  rotation 
of  the  head. 

III.  Lateral  flexion  with  rotation  and  retraction  of  the  neck,  so  that 

the  chin  is  correspondingly  turned  towards  the  opposite  side. 

IV.  Elevation   and   adduction   of   the   shoulder.     Retroflexion   and 

slight  lateral  flexion  of  the  neck,  by  which  the  head,  especially 
when  the  shoulder  is  "fixed,"  is  drawn  to  the  contra-lateral 
side. 
V.  Elevation,  abduction  and  slight  outward  rotation  of  the  shoulder. 
Flexion  of  the  elbow,  at  the  same  time  slight  supination  and 
radial  flexion  of  the  hand.     Only  slight  lateral  and  retro- 
flexion of  the  neck. 
VI.  Moderate   adduction   of   the   shoulder.     Strong  flexion   of   the 
elbow.     Slight  extension  of  the  fingers  and  the  hand,  but  in 
some    individuals    flexion.     Some    supination.     Neck    and 
head  as  in  V. 
VII.  Retraction    and    pronounced    adduction    of    the    shoulder  and 
inward  rotation  of  the  upper  arm.     Extension  of  the  lower 
arm.     Slight  flexion  and  pronation  of  the  hand. 
Slight  flexion  of  the  fingers.     The  shoulder  is  drawn  down. 
Neck  as  in  V. 
VIII.  The  shoulder  is  drawn  down  (latissimus  dorsi).     The  adduction 
of  the  shoulder  is  not  so  strong  as  in  VII.     Inward  rotation 


2i4  DISEASES  OF  THE  SPINAL  CORD 

of  the  arms.     Flexion  and  pronation  of  the  hand.     Flexion 
of  the  fingers  and  the  thumb  with  apposition  of  the  latter. 

Thoracica. 

I.  Retraction  of  the  shoulder.  Slight  lateral  and  retro-flexion  of 
the  neck.  Slight  extension  of  the  arm.  Flexion  and  prona- 
tion of  the  hand.  Flexion  of  the  fingers  and  the  thumb  with 
opposition  of  the  latter.  Usually,  also,  slight  ulnar  flexion 
of  the  hand. 
II.  Retraction  of  the  shoulder.  Slight  flexion  of  the  hand.  Flexion  of 
the  fingers  and  the  thumb  with  opposition  of  the  latter.  In 
some  cases,  slight  pronation  of  the  hand.  Lateral  flexion  of 
the  vertebral  column. 

Postthoracica  (lumbalis) . 

I.  Drawing  in  of  the  abdomen. 
II.  The  same  with  slight  flexion  of  the  hip. 

III.  Drawing  in  of  the  lower  part  of  the  abdomen.    Flexion  of  the  hip. 
TV.  Drawing  in  of  the  lower  part  of  the  abdomen.     Flexion  and 

adduction  of  the  hip.     Extension  of  the  knee. 
V.  Adduction  of  the  hip.     Extension  of  the  knee.     Slight  flexion 

of  the  foot;  slight  extension  of  the  great  toe. 
VI.  Extension  of  the  hip.     Adduction  of  the  thigh.     Strong  flexion 

of  the  knee.     Dorsal  flexion  of  the  foot.     Extension  of  the 

toes.     Adduction  of  the  hallux. 
VII.  Extension  of  the  hip.     Flexion  in  the  knee.     Extension  of  the 

foot.     Rotation  of  the  sole.     Strong  flexion  and  adduction 

of  the  hallux.     Downward  movement  of  the  tail. 
VIII.  Lateral  movement  of  the  tail.     Slight  outward  rotation  of  the 

hip  and  flexion  in  the  knee,  with  extension  in  the  hip  and  foot. 

Strong  flexion  of  the  toes  with  flexion  and  adduction  of  the 

hallux. 
TX.  Lateral  movement  of  the  tail.     Sometimes,  slight  outward  rota- 
tion of  the  thigh  and  adduction  with  flexion  of  the  toes. 
X.  Lateral  movement  of  the  tail  (towards  the  side  of  stimulation) . 

These  discoveries  prove  so  much,  that  the  assumption  of  a  definite  func- 
tional relation  of  the  motor  fibres  united  in  one  anterior  root,  is  not  justified. 
Furthermore,  it  is  noteworthy  that  even  for  paralysis  of  one  muscle,  at  least 
three  successive  anterior  roots  must  as  a  rule  be  destroyed.  In  man,  fre- 
quently, a  still  farther  reaching  anastomosis  of  the  individual  roots  is  as- 
sumed, so  that  beside  the  middle  root,  two  upper  and  two  lower  roots  take 
part  in  the  innervation  of  a  muscle. 


FUNCTIONS  OF  SEGMENTS  OF  THE  SPINAL  CORD         215 

Observers  have  been  largely  occupied,  in  ascertaining  the  localization 
of  the  various  muscles  in  the  individual  spinal-cord  segments,  from  the 
results  of  localized  spinal-cord  lesions.  From  the  wealth  of  schemes  put 
together  for  this  purpose,  we  give  the  one  recently  published  by  Bruns,  as  it 
takes  into  consideration  in  addition  skin  innervation  and  the  reflexes. 

LOCALIZATION    OF    THE  FUNCTIONS    OF   THE  VARIOUS    SEGMENTS    OF   THE 
SPINAL  CORD.     (From  Bruns) 


Segments 


1.  Cervicalis. 


2.  Cervicalis. 


Muscles 


Sensory  innervation  of  the 
skin 


Musculus    rectus    capitis 

posterior  minor.1 
M.  rectus  capitis  posterior 

major. 
M.  obliquus  cap.  superior. 
M.  semispinalis  capitis. 
M.  spinalis      cap.      (pars 

cranialis). 
M.  rectus  cap.  anterior. 
M.  longus  capitis. 
M.  rectus  capitis  lateralis. 
M.  geniohyoideus. 
M.  omohyoideus. 
M.  sternohyoideus. 
M.  thyreo-hyoideus. 
M.  sternothyreoideus. 
One  branch  to  the  muscu- 

1  u  s     intertransversarius 

posterior  cervicalis. 


Reflexes 


M.  rectus  cap.  post,  ma- 
jor. 

M.  obliquus  cap.  inferior. 

M.  semispinalis  capitis. 

M.  spinalis    capitis    (pars 
cranialis). 

M.  longus  atlantis. 

M.  longus  colli. 

M.  longus  capitis. 

M.  geniohyoideus. 

M.  omohyoideus. 

M.  sternohyoideus. 

M.  thyreohyoideus. 
M.  sternothyreoideus. 


1  Cervicales,  1  to  4,  from  Risien  Russel. 


2l6 


DISEASES  OF  THE  SPINAL  CORD 


Segments 


Muscles 


Sensory  innervation  of  the 
skin 


Reflexes 


3.  Cervicalis. 


4.  Cervicalis. 


M.  splenius  capitis  et  cer- 

vicis. 
M.  sternocleidomas- 

toideus. 1 
M.  trapezius. 
Serrations  of  the  musculus 

longissimus  and  the  in- 

tertransversarii. 


Platysma  (Kocher). 

M.  longus  atlantis. 

M.  longus  colli. 

M.  longus  capitis. 

M.  diaphragma. 

M.  scalenus  medius. 

M.  geniohyoideus. 

M.  omohyoideus. 

M.  sternohyoideus. 

M.  sternothyreoideus. 

M.  splenius  capitis  et  cer- 

vicis. 
M.  sternocleidomas- 

toideus. 
M.  trapezius. 
M.  levator  scapulae. 


M.  longus  atlantis. 
M.  longus  colli. 
M.  longus  capitis. 
M.  Diaphragma. 
M.  scalenus  anterior. 
M.  scalenus  medius. 
M.  sternothyreoideus. 
M.  splenius    capitis    et 

cervicis. 
M.  trapezius. 
M.  levator  scapulae. 
M.  rhomboidei. 
M.  supra-   and   infra-spi- 

natus. 
M.  deltoideus. 
M.  biceps  and  coracobra- 

chialis. 
M.  brachialis  int. 
M.  supinator  longus. 


Skin  on  the  back  part  of  the 
head  to  the  vertex;  on  the 
sides  of  the  head  forwards  to 
the  anterior  boundary  of  the 
ear;  skin  over  the  ear;  over 
the  lowest  and  most  poste- 
rior parts  of  the  lower  jaw, 
on  the  neck  and  nape,  at 
the  front  downwards  to  the 
second  rib;  at  the  back 
down  to  the  spina  scapulae 
(according  to  Wichmann, 
the  middle  lower  part  of 
this  dorsal  region  is  sup- 
plied by  the  dorsal  branches 
of  the  fifth  and  sixth  and 
even  seventh  cervical 
nerves).  The  second  cer- 
vical root  reaches  up- 
wards directly  to  the  tri- 
geminus region;  the  third 
innervates  the  neck  and 
nape  down  to  the  clavicle; 
the  fourth  from  there  to  the 
second  rib. 


1  Through  accessorii. 


FUNCTIONS  OF   SEGMENTS  OF  THE  SPINAL  CORD  217 


Segments 


Muscles 


Sensory  innervation  of  the 
skin 


Reflexes 


5.  Cervicalis.        Museums  levator  scapula;. :   Outer  side  of  shoulder,  arm 


6.  Cervicalis. 


M.  teres  minor. 

M.  diaphragma. 

M.  rhomboidei. 

M.  deltoideus. 

M.  biceps  and  coracobra- 
chialis. 

M.  brachialis  internus. 

M.  supra-  and  infra-spi- 
natus. 

M.  supinator  longus  and 
brevis. 

M.  pectoralis  (pars  cla- 
vicularis) .  Possibly  exten- 
sors of  hand. 

M.  serratus  anticus  major. 

M.  splenius. 


and  forearm. 


Scapular  reflex. 

From  the  fifth  cer- 
vical to  the  first 
dorsal  root,  tendon 
reflexes  of  the  mus- 
cles represented  in 
them. 


Outer  parts  of  the  forearm 
on  the  flexor  and  extensor 
surfaces. 


M.  deltoideus. 

M.  teres  major  and  minor. 

M.  biceps  and  brachialis 

internus. 
M.  supra-    and    infra-spi- 

natus. 
M.  supinator  longus   and 

brevis. 
M.  subscapularis. 
M.  pronator   quadratus    Radial  portion  of  the  hand 

and  teres.  and  the  fingers. 

Extensors  and  flexors  of  the 

hand. 

Serratus  anticus  major. 
Possibly  long  extensors  of 

the  fingers. 
M.  splenius. 
M.  scaleni. 


7.  Cervicalis.        Pronators  of  the  hand.  Middle  parts  of  the  forearm 


M.  subscapularis. 

M.  teres  major. 
Extensors  and  flexors  of 

the  hand. 
M.  triceps. 
Pectoralis  major  (pars  cos- 

talis). 
Latissimus  dorsi. 


on  the  flexor  and  extensor 
surfaces. 
Radial  and  median  district 
of  the  hand  and  the  fingers. 


Palmar  reflex. 


Blow  upon  the  pal- 
mar surface  causes 
the  fingers  to  close. 


1  Besides  this,  the  individual  cervical  roots  innervate  also  the  long  muscles,  extending  over 
the  entire  vertebral  column,  at  the  corresponding  level. 


2l8 


DISEASES  OF  THE  SPINAL  CORD 


Segments 

Muscles 

Sensory  innervation  of  the 
skin 

Reflexes 

Long  extensors  and  flexors 

of  the  fingers. 

M.  splenius. 

M.  scaleni. 

Small  muscles  of  the  hand 

(interossei    and    lumbri- 

cales)  ? 

8.  Cervicalis. 

M.  triceps. 

Flexor  carpi  ulnaris. 

Long  extensors  and  flexors 

of  the  hand. 

Interossei  and  lumbricales. 

Scaleni. 

Ulnar  side  of  the  hand,  the 
forearm  and  arm  on  the 
extensor  and  flexor  sur- 
faces.     The   uppermost 
part  of  the  arm  region  is 
supplied  in  addition  by 
dorsalis  2. 

i.  Dorsalis. 

Long  flexors  of  the  fingers. 

Interossei  and  lumbri- 
cales. 

Balls  of  the  thumb  and 
small  finger. 

Scaleni. 

(In  the  eighth  cervical  and 

first  dorsal  root  the  fibres 

for  the  dilatator  pupillae 

pass    to    the    gangliated 

cord.) 

2  to  12  dorsalis, 

The    corresponding    long 

Skin  of  the  chest,  the  back, 

Epigastric     reflex, 

resp.  i  lumba- 

muscles  of  the  vertebral 

and    the    abdomen,  above 

4th~7th  dorsal  root. 

lis. 

column. 

from  the  lower  boundary  of 

M.  serratus  post.  sup.  1-4. 

the  upper  cervical  area,  be- 

M. serratus      post.      inf. 

low  reaching  not  quite  to  the 

Abdominal     reflex, 

9-12. 

inguinal  fold;   skin  of  the 

7th-nth        dorsal 

M.  intercostales       interni 

upper  gluteal  region.    The 

root. 

2-1 1. 

6th  root  reaches  in  front, 

M.  intercostales      externi 

medially,  the  region  of  the 

2-11. 

epigastrium;  the  8th  and  oth 

M.  levat.      cost.      breves 

do  not  reach  beyond   the 

2-1 1. 

skin    over    the    abdominal 

M.  infracostales  2-4  and 

cavity    (nipple   4th   dorsal 

7-9- 

root;  upper  epigastrium  6th, 

M.  levat.  cost,  longi  8-10. 

umbilicus      10th).         The 

M.  transversus  thoracis 

boundaries  of  the  individ- 

3-6- 

ual  skin  regions  pass  hori- 

M. transversus  abdominis 

zontally   about  the  j  trunk; 

7-12. 

therefore,    at    the    thorax, 
over  several  i  ntercostal 
spaces. 

FUNCTIONS  OF  SEGMENTS  OF  THE  SPINAL  CORD 


219 


Segments 

Muscles 

Sensory  innervation  of  the 
skin 

Reflexes 

M.  obliq.  abd.  int.  7-12. 

M.  obliq.  abd.  ext.  7-12. 

M.  rectus  abdominis  8-12. 

M.  pyramidalis  d.  12 

. 

lumb.  1. 

Skin  of  the  inguinal  region, 

Lumbalis  i.1 

Muscles  of  the  abdomen, 

Patellar  reflex  2  nd- 

see  above. 

of  the  uppermost  part  of  the 

4th  lumbaris,  espe- 

Iliopsoas. 

front  and  outer  parts  of  the 

cially  lumb.,  4. 

Sartorius. 

thigh,  skin  of  the  mons  ven- 

Cremaster. 

eris,  the  root  of  the  penis  on 

Quadratus  lumborum. 

the  dorsal  side,  sensibility 
of  the  testicles,  seminal  cord 
and  the   tunica  dartos,  by 
means  of  the  sympathetic. 

Outer  and  front  part  of  the 

Lumbalis  2.1 

Iliopsoas. 

Sartorius. 

hip,  to  the  lower  third  of  the 

Lumbal  plexus. 

Quadriceps  femoris. 
Cremaster. 

thigh. 

Quadratus  lumborum. 

Anterior  portion  of  the  hip; 

Lumbalis  3. 

Small  muscles  of  the  pel- 

vis. 

partly  in  common  with  2, 

M.  iliopsoas. 

innermost  parts  of  the  back 

M.  sartorius. 

of  the  hip  in  its  upper  parts, 

M.  quadriceps  femoris. 

and  the  inner  side  of  the 

M.  adductores  femoris. 

leg. 

M.  quadratus  lumborum. 

Lower  parts  of  the  inner  and 

Lumbalis  4. 

Small  muscles  of  the  pelvis. 

Gluteal    reflex    4th 

M.  quadriceps  femoris. 

anterior  surfaces  of  the  hip, 

and    5th   lumbalis 

M.  adductores  femoris. 

inner  side  of  the  leg,  partly 
also  the  back  inner  margin 
of  the  foot,  excepting  the 
large  toe. 

( ?  probably  lower) . 

Tibialis  anticus. 

(Extensor  digitorum  com- 

mun.,  extensor  hallucis.) 

Anterior  outer  parts  of  the 

Lumbalis  5. 

Tibialis  anticus. 

Extensor  hallucis. 

leg,  outer  and  dorsal  parts 

Extensor  digitorum    com- 

of the  foot,  with  exception  of 

munis. 

the  tips  of  the  toes,  inner 

Musculi  peronei. 

parts  of  the  plantar  surface 
of  the  foot  (with  sacralis  ) . 

1  Perhaps  the  1st  and  2nd  lumbar  segments  contain  no  muscular  nuclei  at  all;  then  the  muscles 
mentioned  here  would  draw  their  nerve  supply  from  the  3d  segment. 


220 


DISEASES  OF  THE  SPINAL  CORD 


Segments 


Muscles 


Sensory  innervation  of  the 
skin 


Reflexes 


Sacral  plexus. 


Sacralis  i  and  2. 


Sacral  plexus. 
Sacralis  3-5. 


M.  flexores  cruris.1 
Musculi  glutaei  (extensores) 

and  abductors  of  the  hip. 
Outward   rotators  of   the 

hip. 
Plantar  flexors  of  the  foot 

(musculature  of  the  calf) 

and  long  flexors  of  the 

toes. 


M.  peronei. 

Muse,  flexoris  cruris. 

Muscul.  glutaei  (extensors) 

and  abductors  of  hip. 
Outward  rotators  of  the 

hip. 
Plantar  flexors  of  the  foot 
Long  flexors  of  the  toes. 
Second    especially:    small 

foot    muscles,    interossei 

and   flexor  digitorum 

brevis. 


Muscles  of  the  perineum 
and  the  transversely 
striated  musculature  of 
the  urethra  (compressor 
urethras)  and  of  the  rec- 
tum (sphincter  ani  exter- 
nus)  and  of  the  sexual 
organs. 


First  sacralis.  Outer  and 
middle  part  of  the  sole, 
heel,  parts  of  the  dorsum  of 
the  foot  (inner  side) ,  tips  of 
the  toes.  Outer  and  back 
part  of  the  calf. 


Second  sacralis.  Middle  part 
of  the  back  of  the  thigh  and 


Third  sacralis.  Skin  over 
the  os  sacrum,  inner  and 
upper  part  of  the  thigh. 


Fourth  and  fifth  sacral  root. 
Perineum,  anus,  and  con- 
centric parts  of  the  skin 
over  the  os  sacrum  close  to 
the  middle  line,  dorsum  of 
penis,  and  anterior  surface 
with  the  exception  of  the 
root  of  the  penis,  skin  of 
the  scrotum. 

(In  the  female,  back  parts 
of  the  large  labia.) 


Plantar  reflex. 


Achilles  tendon  re- 
flex. According  to 
Ziehen,  sacralis  1; 
according  to  Qp- 
penheim,  lumbalis 
5,  sacralis  1  and  2. 

Center  for  erection 
2  and  3  sacralis. 
For  ejaculation  3 
sacralis.2 


Detrusor  and  sphinc- 
ter vesicae,  unstrip- 
ed  musculature  of 
the  rectum  3,  4, 
and   5   sacralis.3 

Sphincter  ani  ex- 
ternus  4  and  5 
sacralis. 

Anal  reflex  5  sa- 
cralis. 


1  The  segments  for  the  flexores  cruris  and  the  glutaei  are  not  yet  clearly  determined.  The 
former  lie  certainly  higher  than  the  latter;  they  may  even  be  below  the  muscles  of  the  calf. 
Both  lie  certainly  below  the  peronei. 

2  According  to  L.  R.  Muller,  not  situated  in  the  conus. 


THE  CONDUCTING  TRACTS  OF  THE  SPINAL  CORD         221 

In  recent  times,  with  the  aid  afforded  by  the  methods  of  chromatolysis  in 
amputations  and  muscle  atrophies,  it  has  been  attempted  to  establish  more 
exactly  the  relations  of  the  individual  columns  of  nuclei  in  the  anterior  horns 
to  the  muscle  groups  of  the  extremities.  According  to  these  investigations,  for 
instance,  every  cell  column  of  the  lumbar  enlargement  seems  to  stand  in  a 
certain  relation  to  all  the  muscles  of  a  limb  segment.  Each  of  these  seg- 
mentary groups  of  nuclei  might  be  divided  into  individual  nuclei,  which  are 
connected  either  with  the  function  of  similar  groups  of  muscles,  or  with 
single  nerves  of  the  limb  segment  concerned.  Finally,  there  might  be  sub- 
divisions here  again  for  individual  muscles.  With  these  investigations  as  a 
basis,  schemes  of  the  various  columns  of  nuclei  in  the  cervical  and  lumbar 
enlargements  with  their  proper  functions  have  already  been  drawn  up, 
though  no  decisive  results  could  be  gained  here. 

If  we  until  now  have  mainly  been  treating  of  the  functions  localized  in 
the  individual  spinal  cord  segments,  we  must  now  turn  to  the  functional 
importance  of  the  conducting  tracts  of  the  spinal  cord.  Their  anatomical 
distribution  we  have  discussed  above. 

The  investigation  of  the  function  of  the  spinal  cord  tracts  has  undergone 
great  changes  with  the  advancing  knowledge  of  their  anatomical  structure. 
Only  since  we  have  been  precisely  informed  as  to  the  course  of  the  individual 
tracts,  which  are  often  mixed  in  the  same  area,  has  it  become  possible  to 
confine  their  experimental  disjunction  to  one  or  a  few  of  them.  Besides,  the 
accurate  microscopic  observation  of  the  point  of  incision  and  the  secondary 
degenerations,  in  which  Marchi's  method  has  been  of  the  greatest  assistance, 
places  in  our  hands  the  indispensable  means  of  controlling  exactly  the  pre- 
arranged injuries  as  to  extent,  as  well  as  the  manifestations  that  follow. 

In  addition  to  the  experimental  investigations  in  higher  mammals,  the 
experience  afforded  by  human  pathology  has  been  of  great  importance. 
In  very  truth,  it  is  indispensable  for  the  ascertainment  of  the  function  of  the 
conducting  tracts  in  man,  since  not  only  in  anatomic  structure,  but  also  in 
the  physiological  value  of  the  individual  tracts,  there  are  important  differences 
between  the  various  classes  of  animals  and  man.  But  one  must  always 
remember,  that  in  the  sense  of  physiological  experiment  pure  disjunctions 
of  definite  divisions  of  the  spinal  cord  in  human  pathology  are  the  rarest 
of  exceptions,  since  pressure  phenomena,  focal  diseases  due  to  other  causes, 
etc.,  are  quite  liable  to  confuse  the  pure  symptoms  of  lost  function. 

Considering  the  gradual  development  of  our  knowledge  of  the  cerebro- 
spinal conducting  tracts,  it  is  easy  to  understand,  how  observers  were  imme- 
diately inclined  to  assign  the  individual  functions  to  the  few  conducting 
tracts  that  were  known.  Thus  the  pyramidal  tract,  especially,  was  long 
considered  as  the  only  pathway  for  transmitting  voluntary  motor  impulses 
from  the  cortex  of  the  cerebrum  to  the  spinal  cord.  This  conception  found 
its  actual  support  in  the  proof  of  descending  degeneration  of  the  pyramidal 


222  DISEASES  OF  THE  SPINAL  CORD 

tract  in  man  after  focal  injuries  to  the  brain,  especially  to  the  inner  capsule 
in  connection  with  a  typical  hemiplegia.  But  it  must  be  borne  in  mind, 
that  in  this  case,  though  only  the  pyramidal  tract  degenerates  down  to  the 
spinal  cord,  it  was  not  alone  interrupted  in  the  brain  by  the  focal  injury, 
but  in  connection  with  the  other  cortico-fugal  tracts  subserving  the  region 
of  the  extremities.  But  even  these  wide  spread  disjunctions  of  the  tracts, 
that  carry  the  motor  impulses,  do  not  prevent  a  considerable  involution  of 
the  hemiplegia  after  some  time. 

An  isolated  disjunction  of  the  pyramidal  tract  is  experimentally  feasible 
only  by  complete  division  of  the  pyramids  in  the  medulla  oblongata,  where 
they  appear  on  the  ventral  surface  and  are  not  mixed  with  other  fibres,  or  by 
section  of  the  pyramidal  crossing  itself  making  a  longitudinal  incision, 
which  at  least  produces,  in  the  dog  and  lower  ape,  complete  disjunction  of 
the  pyramidal  conductivity,  since  in  them  the  anterior  pyramidal  tract  is 
wanting.  In  the  lateral  column  of  the  spinal  cord,  however,  the  pyramidal 
tract  can  be  severed  only  in  conjunction  with  the  rubro-spinal  and  dorso- 
lateral cerebellar  tract. 

While  it  had  been  known  for  some  time,  that  after  severing  the  pyramidal 
tracts  the  motor  effects  in  electric  stimulation  of  the  cortex  of  the  cerebrum 
remain  unchanged,  it  has  recently  been  possible  to  keep  dogs  alive  for  a 
long  time  after  the  pyramidal  tracts  had  been  completely  cut  through. 
Thus  it  was  seen  that  the  animals,  immediately  after  waking  from  the 
narcosis  after  the  operation,  were  able  to  run  about  freely  and,  in  the  use  of 
their  extremities,  could  not  be  differentiated  from  normal  dogs.  In  uni- 
lateral disjunction  of  the  pyramids,  a  certain  awkwardness  in  the  employ- 
ment of  the  muscles  of  the  opposite  side  of  the  body  was  noted  in  perform- 
ing unusual  movements  (waddling);  but  nothing  of  functional  loss  was 
observed.  Correspondingly,  the  isolated  disjunction  of  the  pyramidal 
tracts  does  not  essentially  injure  either  the  tactile  reflex,  passing  over  the 
region  of  the  extremities  of  the  cerebral  cortex,  nor  the  effects  of  electric 
stimulation  of  the  region  of  the  extremities. 

Now,  if  the  second  important  motor  tract  in  the  lateral  column  of  the 
dog  is  disjoined,  the  rubro-spinal  bundle  in  the  medulla  oblongata  above  the 
pyramidal  crossing,  that  is,  before  the  pyramidal  fibres  enter  the  lateral 
column,  there  appears  immediately  clearly  defined  paresis  of  the  limbs  on 
the  same  side,  which,  however,  is  completely  recovered  from  in  the  next 
few  weeks.  In  this,  also,  electric  stimulation  of  the  region  of  the  extrem- 
ities shows  no  deviation  from  the  normal;  the  tactile  reflex,  lost  at  first,  is 
soon  recovered  again  (Fig.  53). 

Not  until  a  disjunction  of  both  the  pyramidal  and  the  rubrospinal  tracts  is 
performed  in  the  lateral  column  of  the  upper  cervical  portion  of  the  cord  of 
the  dog,  are  serious  phenomena  of  loss  of  function  observed.  After  a  tem- 
porary, rather  serious  paralysis  of  the  extremities  of  the  same  side,  a  spastic 


THE  PYRAMIDAL  TRACTS  IX  DOGS  AXD  APES 


223 


paresis  of  the  limbs  remains.  The  tactile  reflex  is  permanently  lost;  iso- 
lated movements  of  the  extremities  are  no  longer  observed.  Above  all,  the 
effect  of  the  electric  stimulation  of  the  crossed  region  of  the  extremities  is 
absolutely  lost.  In  the  dog,  therefore,  the  impulses  of  the  cerebral  cortex 
are  transmitted  to  the  spinal  cord  exclusively  by  way  of  the  direct  cortico- 
spinal (pyramidal)  and  the  rubro-spinal  tracts. 

In  the  lower  apes  also  the  total  destruction  of  the  pyramidal  crossing  has 
the  surprising  result,  that  no  paralysis  of  the  extremities  appears,  not  even 
immediately  after  the  operation.  But  not  only  the  coarser  voluntary  move- 
ments, as  they  appear  in  the  acts  of  running  and  climbing,  are  retained,  but 
also  the  delicate  isolated  movements  of  the  fingers  which  are  necessary,  for 
instance,  in  reaching  for  small  pieces  of  food,  are  absolutely  intact.  On 
the  whole,  however,  the  ape.  in  whom  the  pyramid  has  been  cut,  performs  all 
his  movements  more  slowly  than  the  normal  ape,  and  thus  makes  altogether 
a  clumsier  impression  (Fig.  54). 


Fig. 


-Division  of  the  right  postero-lateral  column  including  the  rubro-spinal  bundle  at  the 
height  of  the  pyramidal  crossing  in  the  dog.     Marchi  preparation. 


But  that  the  pyramidal  tracts  are  of  considerably  greater  importance 
in  the  ape  than  in  the  dog,  is  proved  by  the  result  of  cerebral  cortex  stimula- 
tion; when  this  is  performed  several  weeks  after  the  disjunction  of  the  py- 
ramidal tracts,  only  two  small  fields  of  the  region  of  the  extremities,  those 
corresponding  to  the  centers  for  finger  and  toe  movements,  are  proven 
electrically  excitable,  whereas  all  the  rest  of  the  field  does  not  respond. 

Nor  does  the  isolated  disjunction  of  the  rubrospinal  tract  of  the  lateral 
column,  before  it  joins  with  the  pyramidal  tract,  lead  to  any  paresis,  worth 
mentioning,  of  the  extremities  on  the  same  side.  In  unilateral  disjunction 
they  are  somewhat  more  awkward  for  a  few  days  than  the  extremities  of  the 
other  side.  But  after  a  few  days  they  are  used  quite  normally  for  all 
purposes,  including  the  most  delicate  grasping  movements,  with  the  excep- 
tion that  the  normal  arm  is  somewhat  preferred.     To  this  corresponds  also 


224  DISEASES  OF  THE  SPINAL  CORD 

the  completely  normal  electric  excitability  of  the  crossed  region  of  the  ex- 
tremities in  the  cerebral  cortex. 

Now,  if  in  the  ape,  both  pyramidal  and  rubro-spinal  tracts  are  interrupted 
together,  by  complete  division  of  the  postero-lateral  column  in  the  third 
cervical  segment,  the  result  differs  materially  from  that  obtained  in  dogs. 
On  the  first  two  days  after  such  an  operation  the  extremities  concerned  hang 
down  limply,  and  are  but  slightly  used  in  the  general  movements.  But  in 
the  next  few  days,  the  motor  power  of  the  extremities  gradually  increases; 
arms  and  legs  are  used  not  only  in  general,  but  possibly  even  better  in  isolated 
movements.  Indeed,  after  a  week  the  motor  weakness  is  virtually  wholly 
adjusted.     After  special  practice  (the  sound  arm  is  bound  down  for  some 


Fig.  54. — Division  of  the  pyramidal  crossing  in  the  ape  (Macacus  rhesus) .     Marchi  preparation. 


time  every  day)  the  ape  prefers  this  arm,  even  for  grasping.  In  the  ape,  in 
contradistinction  to  the  dog,  and  still  sharper  contrast  to  man,  no  spastic 
phenomena  follow  such  lateral  column  disjunction. 

It  is  in  full  accord  with  this  result,  that  in  the  ape  the  electrical  stimula- 
tion of  the  region  of  the  extremities  several  weeks  after  a  unilateral  disjunc- 
tion in  the  postero-lateral  column  still  transmits  motor  impulses  to  the  spinal 
cord,  which,  though  they  are  virtually  confined  to  the  fields  of  the  hand  and 
foot  region,  are  still  somewhat  more  marked  than  may  be  observed  in  bilat- 
eral isolated  pyramidal  division. 

Since,  then,  the  combined  disjunction  of  the  cortico-spinal  and  the 
rubro-spinal  tracts  in  the  ape  leads  only  to  a  slightly  greater  disturbance  of 
the  voluntary  movements  of  the  extremities,  than  the  isolated  disjunction  of 
each  of  these  tracts,  other  tracts  must  be  involved  in  their  conduction  to 
the  spinal-cord  centers.  In  the  first  place  it  can  easily  be  proven  that  it 
is  not  only,  as  one  might  think,  the  corresponding  conducting  tracts  of  the 
other  half  of  the  spinal  cord  which  permit  the  impulses  to  cross  over  to  the 
injured  side  at  the  level  of  the  corresponding  anterior-horn  centers.  Even 
in  the  dog,  bilateral  disjunction  of  the  postero-lateral  column  leads  to  no 
serious  disturbance  so  far  as  the  usual  locomotion  is  concerned.     In  apes, 


SECTION  OF  THE  AXTERIOR  COLUMNS  IN  DOGS 


also,  to  the  complete  division  of  one  postero-lateral  column,  a  complete 
division  of  the  other  may  be  added  after  2  to  3  weeks,  and  still  the  ape  can 
learn  to  run  and  climb  again  after  a  few  days,  and  is  able,  though  with 
lessened  vigor,  to  reach  for  food.  Furthermore,  even  if  in  the  ape  first  the 
entire  arm  region  of  one  hemisphere  of  the  cerebrum  is  removed  to  the 
extent  marked  out  by  Munk,  and  the  isolated  grasping  movements  of  the 
opposite  arm  are  thereby  lost  by  complete  destruction  of  the  corresponding 
pyramidal  tract,  and  to  this  is  added,  after  a 
few  weeks,  the  disjunction  of  the  postero-lateral 
column  of  the  same  side  in  the  third  cervical 
segment,  which  has  been  innervating  the  here- 
tofore normal  extremities,  he  can  nevertheless 
soon  learn  to  use  these  extremities  again  for 
general  movements  and  isolated  grasping  mo- 
tions. Indeed,  the  disjunction  of  the  impulses 
of  the  other  side  can  be  carried  still  farther,  by 
cutting  through  the  same  ape's  other  postero- 
lateral column  in  the  first  cervical  segment, 
thus  disjoining  the  one  remaining  rubro-spinal 
bundle  and  the  few  uncrossed  pyramidal  fibres 
which  radiate  into  this  column,  only  to  find  that 

the  restitution  of  the  arm,  which  still  has  its  FlG>  55._Combined  disjunc- 
cerebral  cortical  centers,  is  not  impeded,  though  tion  of  one  arm  region  and  both 

.         « postero-lateral  columns.     I,     Ex- 
the    motor  lateral  column   tracts   On    both  Sides    tirpation   of   the   left   arm  region 

are  totally  disjoined.  These  movements  of  the  "  £f /»«^.  £SSaS 
extremities  concerned  are  present  even  though  column  and  of  parts  of  the  anterior 

,  .  .      M   J  ,.      .      ,  .  ,.      column  in  the  third  cervical  seg- 

only  at  first  to  a  very  limited  extent,  lmmedi-  ment;  in,  complete  division  of 
atelv   after   complete    division  of  the   postero-  ^e  ^ht  postero-lateral  column  in 

J  *■  x  the  first  cervical  segment. 

lateral  columns,  so  that  an  absolute  restitution 

should  not  be  spoken  of,  as  part  of  the  motor  impulses  must  already  be 
passing  normally  outside  of  the  motor  lateral  column  tracts  of  the  spinal 
cord  (Fig.  55). 

Here,  then,  the  anterior  columns  must  be  considered;  in  them  we  have 
already  met  with  (see  above)  a  series  of  cerebro-spinal  tracts  passing  to  the 
spinal  cord.  Older  physiologists  already  ascribed  to  them  the  most  impor- 
tant share  in  the  conduction  of  movements.  But,  nevertheless,  they  were 
later  neglected  as  compared  with  the  pyramidal  tract,  inasmuch  as  even 
human  pathology  furthered  the  knowledge  of  the  motor  functions  of  the 
anterior  columns  but  slightly.  Isolated  disjunction  of  the  anterior  columns 
may  be  performed  in  dogs,  from  the  front,  along  the  lower  margin  of  the  atlas, 
directly  beneath  the  pyramidal  crossing.  Such  an  isolated  disjunction  of 
the  anterior  columns  leads  to  no  paralysis  of  the  extremities.  On  the  other 
hand,  the  anterior  columns  subserve  a  function  especially  important  for  the 


226  DISEASES  OF  THE  SPINAL  CORD 

station  of  the  animal,  namely,  the  innervation  of  the  musculature  of  the 
back.  The  dog,  deprived  of  his  anterior  columns,  runs  in  a  wobbly  fashion, 
with  legs  apart  and  with  the  back  part  of  his  body  shaking  from  side  to  side. 
He  can  not  turn  sideways  in  a  normal  way,  and  when  he  is  put  on  the  table 
with  the  back  part  of  his  body  hanging  down  over  the  edge,  he  can  not  raise 
it  as  a  normal  dog  can.  Electric  cerebral  cortex  irritation  of  the  regions  of 
the  extremities,  however,  does  not  show  the  slightest  loss  of  function,  after 
the  anterior  column  has  been  disjoined. 

In  the  dog,  therefore,  neither  the  disjunction  of  the  lateral,  nor  that  of 
the  anterior  columns,  brings  about  paralysis  of  the  extremities,  though 
under  normal  conditions  the  motor  impulses,  which  leave  the  cerebrum, 
according  to  the  results  of  stimulation  of  the  cerebral  cortex,  seem,  on  the 
whole,  to  use  the  lateral  column.  Since,  on  the  other  hand,  the  disjunction 
of  the  pyramidal  tract  together  with  the  anterior  column,  stops  neither  the 
motor  activity  of  the  extremities,  nor  the  effect  of  stimulation  of  the  cerebral 
cortex,  we  reach  the  important  conclusion,  for  the  dog  at  least,  that  all  these 
motor  lateral  column  and  anterior  column  tracts  can  mutually,  to  a  great  extent, 
replace  each  other,  with  the  exception  that  the  impulses  for  the  isolated 
movements,  dependent  exclusively  on  the  cortex  of  the  cerebrum,  use  the 
lateral  column  tracts  par  preference. 

In  the  monkey,  an  isolated  disjunction  of  the  anterior  column  has  not  yet 
been  attempted.  It  has,  however,  been  ascertained,  that  in  the  ape  also  a 
destruction  of  the  anterior  column  together  with  the  pyramidal  tract  does 
not  cause  any  loss  of  motor  function  of  the  extremities  concerned,  nor  a 
total  abolition  of  the  electric  cortical  excitability,  so  that  the  rubro-spinal 
tract  alone,  might,  even  in  the  ape,  suffice  to  conduct  these  impulses.  But 
there  can  be  no  doubt,  according  to  the  above  quoted  results  of  disjunction 
of  the  lateral  column,  that  the  anterior  column  possesses  in  the  ape  a  higher 
physiological  value  than  in  the  dog,  since  it  is  used  in  great  measure  also 
for  the  conduction  of  the  isolated  movements  dependent  on  the  cerebral 
cortex,  even  to  the  most  delicate  play  of  the  fingers. 

But  now,  if  in  the  dog  or  monkey  the  entire  motor  conduction  of  half  the 
spinal  cord  is  disjoined,  either  by  induction  of  a  complete  hemilesion,  or 
by  severing  only  the  anterior  and  lateral  columns  together  in  the  upper 
cervical  portion,  it  appears  that  even  then  motor  impulses  from  the  other 
half  of  the  spinal  cord  may. reach  the  spinal  centers  of  the  extremities  con- 
cerned. Thus,  after  transient  serious  paralysis  a  moderate  degree  of 
movement  is  regained,  even  though  the  excitability  of  the  opposite  cerebral 
hemisphere  is  lost,  and  in  the  monkey,  at  least,  the  grasping  movement  of 
the  affected  arm  remains  seriously  injured. 

The  remarkable  power  of  restitution  which,  with  respect  to  coarse 
locomotion,  is  innate  in  the  spinal  cord,  is  best  demonstrated  by  an  experi- 
ment on  the  cat,  in  which  the  motor  tracts  of  the  lateral  column,  the  medial 


THE  MOTOR  SPINAL  CORD  TRACTS  IN  APE  AND  MAN    227 

halves  of  the  anterior  columns,  and  in  addition  the  posterior  columns  on 
both  sides,  were  destroyed.  In  spite  of  the  fact  that  the  cat  was  totally 
paralyzed  in  all  four  extremities  for  2  1/2  weeks,  ordinary  walking  move- 
ments gradually  returned,  which  improved  to  such  an  extent  in  the  course  of 
1  1/2  months,  that  the  cat  was  able  to  reach  by  jumping,  and  to  bring  down 
meat  from  the  edge  of  a  table  of  ordinary  height.  For  this  action,  which 
certainly  demands  a  considerable  amount  of  strength  and  agility,  the 
lateral  remnants  of  the  anterior  columns  sufficed  in  this  case,  though  the 
extensive  destruction  of  sensory  fibres  had  certainly  increased  the  difficulty 
of  restitution. 

In  the  anthropoid  ape,  experiments  concerning  the  functions  of  the  motor 
conducting  tracts,  which,  as  is  easily  understood,  would  be  of  especial  value 
particularly  because  of  the  far  reaching  analogy  of  its  anatomic  structure 
with  that  of  man,  have  been  carried  out  only  in  isolated  cases.  The  destruc- 
tion of  the  smaller  medial  half  of  one  pyramid  immediately  above  the  pyram- 
idal crossing,  is,  in  the  chimpanzee,  followed  by  slight  weakness  in  the  arm 
concerned,  without  decided  phenomena  of  paralysis,  without  any  indication 
of  spasms,  and  with  retention  of  the  delicate  grasping  movements  of  the 
fingers.  A  destruction  of  the  medial  half  of  one  anterior  column,  including 
the  anterior  pyramidal  tract,  which  in  the  chimpanzee  already  appears  in 
the  second  cervical  segment,  caused,  likewise,  but  a  slight  tendency  to 
spare  tfre  extremities  on  the  same  side,  especially  the  arm,  without  the  least 
disturbance  even  of  the  most  delicate  finger  movements. 

Concerning  the  functions  of  the  motor  spinal  cord  tracts  in  man,  it  was 
supposed,  up  to  most  recent  times,  that  the  pyramidal  tract  represented  the 
path  of  voluntary  movements  and  that  its  destruction  was  necessarily 
accompanied  by  paralysis  of  the  corresponding  extremities,  with  spasms  and 
exaggeration  of  the  tendon  reflexes.  If  in  hemiplegias,  the  result  of  lesions 
in  the  internal  capsule,  a  return  of  voluntary  movement  could,  as  a  rule, 
be  noted  after  a  shorter  or  longer  period  of  time,  this  was  supposed  to  be 
due  to  the  fact  that  part  of  the  pyramidal  fibres  were  preserved.  But  after 
the  possibility  of  almost  complete  functional  replacement  of  the  pyramidal 
tracts  had  been  proved  by  experiments  on  animals,  especially  by  those  on 
such  monkeys,  which  in  the  structure  of  their  nervous  system  and  in  the 
functions  of  their  extremities,  especially  the  arms,  resembles  man,  a  revision 
of  the  former  theory  in  man  was  also  unavoidable.  The  exact  study  of 
cerebral  hemiplegias  proved,  that  in  spite  of  a  totally  destroyed  pyramidal 
tract,  an  extensive  restitution  of  the  motor  function  might  occur,  which,  if 
the  defects  be  acquired  in  earliest  childhood,  may  even  approach  the  normal 
conditions.  Even  in  spinal  hemiplegia,  due  to  total  destruction  of  one 
lateral  column  in  the  spinal  cord,  there  is  a  restitution  of  approximately  the 
same  extent.  In  bilateral  loss  of  the  lateral  pyramidal  tract,  as  it  has  been 
observed  in  approximately  pure  form  in  some  cases  of  "spastic  spinal  pa- 


228  DISEASES  OF  THE  SPINAL  CORD 

ralysis,"  there  is  no  serious  paralysis,  but  only  a  serious  hindrance  in  walking 
because  of  rather  severe  spasms  in  the  legs. 

As  all  these  results  prove  with  certainty  that  the  loss  of  the  pyramidal 
tracts  is  not  followed,  in  man,  by  an  irreparable  loss  of  voluntary  movements, 
there  remain  only  as  consequences  of  the  loss  of  the  pyramidal  tracts,  exag- 
gerated tendon  reflexes  with  clonus,  as  well  as  the  Babinski  toe  reflex  and 
spasms.  Whether  the  latter  are  an  absolutely  necessary  result  of  the  loss 
of  pyramidal  functions  is,  to  say  the  least,  questionable.  In  human  path- 
ology cases  without  injury  of  the  pyramidal  tracts  have  been  observed  with 
severe  spasms,  as  well  as  other  cases  in  which  diseases  of  the  pyramidal 
tracts  were  present  with  scarcely  a  suggestion  of  spasms.  But  it  must  be 
granted,  that  the  combination  of  disease  of  the  pyramidal  tract  and  muscle 
tonus  increased  to  spasm  represents  the  rule. 

But  even  though  the  pyramidal  tract  is  unquestionably  capable  of  a  con- 
siderable measure  of  replacement  in  man  too,  still  its  importance  is  far 
greater,  than  it  is  in  all  animals  up  to  apes,  and  it  seems  practically  certain, 
that  sudden  isolated  interruption  in  the  conductivity  of  the  pyramidal  tracts,  as 
occurs  in  rare  cases  in  the  medulla  oblongata,  is  immediately  followed  by 
serious  paresis. 

Considering  now  the  question  of  compensation  tracts  for  the  pyramidal 
tracts  in  man,  the  rubrospinal  bundle  seems  to  have  become  so  rudimentary 
here,  that  it  can  scarcely  be  considered  in  the  restitution  of  the  motor  func- 
tion. So  far  as  the  anterior  pyramidal  tract  is  concerned,  which,  of  course, 
escapes  in  spinal  diseases  of  the  lateral  column,  and  might  possibly  be  of 
importance  for  motor  conductivity,  nothing  certain  is  yet  known  about  its 
function.  We  know  of  no  difference  between  the  symptoms  of  total  destruc- 
tion of  the  pyramidal  tract  above  the  crossing,  and  a  lesion  of  the  lateral 
column,  with  the  anterior  pyramidal  tract  intact.  The  above  mentioned 
experiment,  in  which  the  anterior  pyramidal  tract  of  one  side,  was  disjoined 
in  the  chimpanzee,  seems  to  make  it  apparent,  that  it  is  not  connected  in  any 
way  with  the  other  half  of  the  spinal  cord.  This  leaves  the  other  anterior 
motor  tracts  to  compensate  for  the  pyramidal  tract,  analogous  to  the  well 
established  relations  in  the  lower  ape.  Furthermore,  pathology  proves 
definitely,  that  in  extensive  destruction  of  the  anterior  tracts,  the  conduc- 
tivity through  the  lateral  pyramidal  tracts  suffices  for  the  retention  of 
motility. 

And  finally  in  man  also,  after  destruction  of  one  entire  half  of  the  spinal 
cord,  the  motor  conductivity  of  the  other  side  may  bring  about  considerable 
recovery,  especially  for  the  leg  of  the  injured  side.  In  spite  of  initial  serious 
paralysis  of  the  leg,  such  individuals  can,  months  and  years  afterwards, 
take  long  walks  without  assistance.  Insufficient  observations  for  satis- 
factory conclusions  relative  to  this  matter  have  been  made  concerning  the 
arm.     ■ 


FUNCTION  OF  ASCENDING  TRACTS  OF  SPINAL  CORD      229 

Since,  therefore,  the  pyramidal  tract  can  be  replaced  almost  completely 
in  higher  mammals,  and  very  largely  in  man,  by  means  of  the  other  motor 
spinal  cord  tracts,  so  that  it  is  not  possible  to  ascribe  to  it  a  specific  function, 
this  question  arises :  of  what  importance  is  the  greater  and  greater  develop- 
ment, of  such  an  uninterrupted  cortico-spinal  tract  in  the  ascending  animal 
series?  The  supposition,  that  this  direct  connection  between  the  cerebral 
cortex  and  the  spinal  cord  is  necessary  for  the  individual's  learning  of  special 
motions,  is  contradicted  by  the  established  fact,  that  the  ape,  even  after 
being  deprived  of  his  pyramidal  tracts,  can  be  taught  new  and  complicated 
arm  movements.  Since  the  cerebral  cortex  can  influence  the  spinal  centers 
through  the  pyramidal  tracts  only  without  the  assistance  of  the  subcortical 
brain  centers,  it  is  probable,  on  the  contrary,  that  in  the  learning  of  new 
movements,  the  influence  of  these  latter  centers  upon  the  gray  matter  of  the 
spinal  cord  is  necessary  for  clearing  the  path  of  the  groups  of  ganglion  cells 
in  the  anterior  horns,  which  are  essential  to  this.  But  when  the  movement 
is  completely  controlled,  the  direct  transference  of  the  impulse  from  the 
cerebral  cortex  to  the  spinal  cord,  with  its  anterior  horn  cells  already  ar- 
ranged for  the  definite  combinations  of  muscles,  suffices  and  the  centers  of 
the  mid-  and  hind-brains  may  be  passed  over.  The  pyramidal  tract  is, 
accordingly,  the  real  conducting  tract  for  the  immense  number  of  movements 
already  acquired;  the  cerebro-spinal  tracts,  on  the  other  hand,  that  are 
interrupted  in  the  mid-brain,  with  their  relations  to  the  cerebellum,  are  of 
the  utmost  importance  in  learning  movements. 

In  turning  now,  to  the  function  of  the  ascending  tracts  of  the  spinal  cord, 
we  shall  first  consider  the  disturbances,  which  are  caused  by  complete  eli- 
sion of  all  the  sensory  impulses  of  an  extremity  passing  through  the  posterior 
roots  to  the  spinal  cord.  For  instance,  if,  in  the  ape,  we  cut  away  all  the  poste- 
rior roots  concerned  with  the  arm,  from  the  fourth  cervical  to  the  fourth 
dorsal  segments,  there  is  not  only,  as  is  to  be  expected,  total  loss  of 
all  sensory  conductivity;  but  the  grasping  movements  of  the  arm  concerned 
also  disappear  at  first.  It  was  therefore  supposed,  that  through  this  injury 
the  isolated  movements  dependent  on  the  region  of  the  extremities  in  the 
cortex  were  being  seriously  impaired,  or  even  destroyed  with  rather  slight 
injury  to  the  general  movements,  thereby  proving  the  necessity  of  the  impulses 
carried  from  the  skin  and  muscles  to  the  central  organ  for  the  execution  of 
the  most  highly  organized  movements.  But,  in  contradistinction  to  this, 
the  most  recent  investigations  have  shown,  that  in  fact,  all  normal  movements 
of  the  extremities  are  injured  by  the  loss  of  all  the  centripetal  impulses.  If 
one  avoids  all  collateral  injuries  of  the  spinal  cord,  the  disturbances  of  the 
general  movements,  which  are  dependent  on  the  subcortical  centers,  remain 
unchanged.  The  grasping  movements,  however,  which  are  principally  or 
entirely  dependent  on  the  centers  of  the  cerebral  cortex,  improve  under  the 
influence  of  practice;  the  loss  of  the  stimulations,  that  normally  pass  to  the 


23o  DISEASES  OF  THE  SPINAL  CORD 

region  of  the  extremities  through  the  sensory  tracts,  is  extensively  compen- 
sated for  by  the  other  sensory  impulses,  especially  those  of  the  visual  sense. 
Concerning  the  function  of  the  individual  ascending  conducting  tracts  of 
the  spinal  cord,  the  conducting  paths  for  the  various  forms  of  sensation  must 
be  regarded  separately,  whether  one  assumes  now  that  they  are  specifically 
differentiated  from  the  periphery,  or  that  they  acquire  their  peculiar  charac- 
teristics only  with  the  aid  of  the  spinal  centers.  We  must  therefore  consider 
separately : 

i.  The  tract  for  pain  sensations. 

2.  The  tract  for  temperature  sensations,  in  which  sensations  of  cold 
and  those  of  heat  must  be  separately  regarded. 

3.  The  tract  for  pressure  sense. 

4.  The  tract  for  tactile  sensations. 

5.  The  tract  for  deep  sensibility  (bathycesthesia) ,  among  the  com- 
ponents of  which  muscle  sense  with  position,  feeling,  sensations 
of  movement,  etc.,  are  most  important. 

Just  as  with  motor  spinal  cord  tracts,  observers  have  endeavored  to 
assign  to  each  one  of  the  known  centripetal  conducting  tracts  a  function 
peculiar  to  itself  alone.  But  here,  too,  it  has  been  established  with  the 
increase  of  anatomic  knowledge  and  the  refinement  of  physiological  investi- 
gation, more  and  more  clearly,  that  several  tracts  may  share  the  same  func- 
tion, and  to  a  great  extent,  compensate  for  one  another.  Unfortunately, 
however,  animal  experiments  relative  to  the  conduction  of  sensibility,  suffer 
from  the  difficulty  in  separating  clearly  the  conscious  sensations,  rising  in 
the  cerebral  cortex,  from  the  general  feelings  evoked  in  the  deeper  centers 
of  the  brain. 

The  conduction  of  pain  sensation  in  the  dog  and  monkey  is  mostly  given 
over  to  the  lateral  columns;  their  disjunction  lowers  it  considerably,  though 
it  does  not  wholly  destroy  it.  It  is  certain,  at  the  same  time,  that  the  majority 
of  the  fibres  concerned  in  the  sense  of  pain,  after  their  entry  into  the  gray 
matter  of  the  spinal  cord,  and  especially  of  the  posterior  horn,  crosses  to 
the  other  half  of  the  spinal  cord,  without,  however,  in  the  higher  mammals  a 
complete  crossing  of  pain  conductivity  taking  place.  Disjunction  of  one  lat- 
eral column,  therefore,  removes  the  pain  sensation  on  neither  side,  but  lowers 
it  very  markedly  temporarily  on  the  opposite  half  of  the  body.  One  can 
prove,  furthermore,  that  the  conduction  of  pain  in  the  lateral  column  is, 
on  the  whole,  confined  to  its  ventral  half.  When  the  lateral  columns  are 
conserved,  on  the  other  hand,  disjunction  of  the  balance  of  the  spinal  cord 
columns  leads  to  no  disturbance  of  pain  sensation.  After  destruction  of  the 
lateral  columns  in  the  dog,  the  preserved  remainder  of  pain  conduction,  with 
which  no  exact  localization  is  connected,  and  which  probably  represents 
only  the  subcortical  components  of  the  sensations  of  pain,  just  as  it  is  retained 


TRACTS  FOR  PAIN,  TEMPERATURE  AND  PRESSURE        231 

after  the  extirpation  of  the  cerebrum,  is  in  no  way  influenced  by  the  dis- 
junction of  the  posterior  columns.  On  the  other  hand,  the  additional  dis- 
junction of  the  anterior  columns  in  the  upper  cervical  portion  of  the  cord 
leads  to  an  almost  total  loss  of  the  pain  sensation.  But  even  now,  if  all  the 
white  spinal  cord  columns  are  severed  at  various  levels  of  the  cord,  there 
still  remains  a  remnant  of  pain  sensation,  induced  by  strongest  stimulations. 

If,  therefore,  the  antero-lateral  columns  must  be  considered  the  most 
important  tract  in  the  conduction  of  pain  sensations,  it  is,  nevertheless  true, 
that  the  anterior  columns  are  also  concerned,  in  this  function,  even  if  but 
slightly,  or  are  at  least  able  to  assume  this  function  by  substitution.  Finally, 
there  must  be  considered  the  possibility,  even  if  it  be  but  a  very  slight  one, 
of  the  sensations  of  pain  being  conducted  through  the  short  paths  of  the 
gray  matter.  This  conception  corresponds  to  the  older  discoveries  in  the 
rabbit,  that  the  conduction  of  pain  is  not  interrupted  by  a  unilateral  lesion 
of  the  spinal  cord  in  a  definite  segment,  and  a  second  lesion  of  the  other 
half  of  the  spinal  cord  a  few  segments  higher  up.  In  the  ape,  on  the  other 
hand,  such  a  bilateral  hemisection  of  the  spinal  cord  leads  to  complete  loss 
of  sensibility. 

Since  the  discovery  of  Gower^s  ventral  spino-cerebellar  tract  in  the  antero- 
lateral column,  the  chief  conduction  of  the  sensation  of  pain  has  been  fre- 
quently ascribed  to  this  tract.  But  since  we  know  that  Gower,s  tract  ends 
in  the  cerebellum  and  that  total  extirpation  of  the  cerebellum  does  not  result 
in  any  disturbance  of  the  pain  sensation,  this  view  must  be  rejected  as  irrecon- 
cilable with  the  facts.  In  the  conducting  path  for  the  sensation  of  pain  in 
the  anterior  column  no  continuous  tract  is  concerned,  but  there  is  a  chain 
of  shorter  and  longer  intersegmental  nerve  tracts,  always  returning  to  the 
gray  substance  of  the  spinal  cord,  the  longest  fibres  of  which,  might,  of  course, 
commingle  with  those  of  Gower's  tract  (Fig.  56). 

Concerning  the  temperature  sense,  its  conduction  in  the  lateral  column 
corresponds  in  the  main  with  that  of  the  pain  sensation.  In  the  monkey 
there  seems  to  be  a  bilateral  conduction,  showing  a  preference  for  the  crossed 
side.  But  new  experiments,  carried  out  by  Kalischer's  training  methods, 
have  proved,  that  in  the  dog  the  conscious  temperature  sensations  are  through- 
out their  course  crossed.  The  training,  connected  with  the  temperature 
sense,  was  lost  after  severing  of  the  crossed  half  of  the  spinal  cord. 

The  conduction  of  the  pain  sense  is  paralleled  by  that  for  the  pressure 
sense;  when  pressure  is  in  any  way  increased,  it  is  easily  transformed  into 
a  pain  sensation.  Here,  too,  the  main  conducting  path  lies  in  the  lateral 
column  with  preponderance  of  the  crossed  conduction  in  the  spinal  cord. 
In  destruction  of  a  lateral  column  in  the  dog,  an  increase  of  the  pressure 
sense  of  the  same  side  is  often  noted ;  it  is  accompanied  also  by  a  hyperal- 
gesia. On  the  other  hand,  disjunction  of  the  posterior  column  seems  in 
no  way  to  influence  the  pressure  sense. 


232 


DISEASES  OF  THE  SPINAL  CORD 


So  far  as  the  tactile  sensation  is  concerned,  it  is  naturally  dependent  upon 
a  certain  degree  of  pressure  sense,  and  is  lost,  therefore,  when  the  other  is 
markedly  lowered,  as  after  bilateral  disjunction  of  the  lateral  column.  But, 
on  the  other  hand,  with  retained  or  even  increased  pressure  sense,  the  delicate 
localized  tactile  sensations  may  be  severely  lowered,  or  altogether  lost.     The 


Fig.  56.— Conduction   of   the   pain  Fig.  57. — Conduction  of  the  touch  sensation 

sensation  in  the  spinal  cord,     a,  Crossed  in  the  spinal  cord.    H,  Posterior  column;  V, 

antero-lateral  column^  b,  antero-lateral  anterior  column, 
column  at  the   same   side;  c,  crossed 
anterior  column;  p,  posterior  root. 

conduction  of  the  tactile  sensations  has  been  previously  credited  exclusively 
to  the  long  posterior  column  tracts,  which,  without  interruption  ascend 
from  the  periphery  and  the  spinal  ganglia  to  the  nuclei  of  the  medulla  oblon- 
gata and  possess  a  secure  connection  with  the  cerebral  cortex  by  way  of  the 
lemniscus  tract  and  the  optic  thalamus.  According  to  previous  investiga- 
tions, total  division  of  the  posterior  column  in  the  dog  was  said  to  destroy 
the  tactile  sensation,  whereas,  on  the  other  hand,  in  abnormal  heightening 


TACTILE  AND  MUSCLE  SENSE  233 

of  the  sensibility,  due  to  serious  loss  of  blood,  a  rabbit,  whose  entire  cord 
was  divided  with  exception  of  the  posterior  column,  still  reacted  to  slight 
touch  of  the  hind  feet  with  movements  of  his  head  and  ears.  New  experi- 
ments, on  a  dog,  in  which  the  total  division  of  the  posterior  column,  without 
additional  injury,  was  established  by  exact  microscopic  investigation,  have 
proved,  however,  that  the  tactile  sensations  are  retained  intact  after  total  dis- 
junction of  the  posterior  cord.  Nor  does  an  extension  of  the  unilateral  division 
of  the  posterior  column  to  the  posterior  horn  and  the  extreme  dorsal  layer 
of  the  lateral  column  destroy  tactile  sensations  in  the  extremities  of  the  side 
concerned.  If  in  the  normal  dog,  one  severs  the  anterior  column  in  the 
first  cervical  segment,  the  tactile  sensation  is  at  first  lowered,  but  soon 
returns  to  its  normal  degree.  But  if  total  division  of  the  posterior  column 
is  combined  with  that  of  the  anterior  column  in  the  upper  cervical  portion  of 
the  cord,  the  tactile  sensations  are  completely  lost,  and  the  tactile  reflexes 
disappear  in  spite  of  the  intactness  of  the  lateral  column.  At  the  same  time 
the  ordinary  pressure  sense  and  the  pain  sensations  are  in  nowise  disturbed. 

There  exists,  therefore,  a  double  conducting  path  for  the  tactile  sensations, 
that  through  the  posterior  column  on  the  same  side,  and  that  through  the 
anterior  column  (probably  only  crossed).  The  anterior  column  tract  of 
the  tactile  sensation  must  be  built  up  in  the  spinal  cord  from  shorter  inter- 
segmental tracts,  since  long  anterior  column  fibres,  ascending  to  the  medulla 
oblongata  from  the  lower  spinal  cord  segments  are  not  known.  Both  tracts 
are  able  to  immediately  replace  each  other,  and  both  might,  therefore,  be 
normally  used  (Fig.  57). 

For  the  muscle  sense  as  well,  the  most  important  components  of  which 
are  the  sense  of  position  and  the  co-ordination  of  movements,  the  posterior 
column  was  oftenest  considered  as  the  path  of  the  conduction.  But  here  too 
the  isolated  posterior  column  section  in  the  dog  results  in  no  disturbance 
of  the  functions  mentioned.  In  most  recent  times  observers  have  given 
special  attention  to  the  spino-cerebellar  lateral  column  tracts,  since  unques- 
tionably a  large  number  of  sensations  stream  to  the  cerebellum  from  the 
domain  of  the  deep  sensibility,  which  are  used  up  for  the  more  delicate 
regulation  of  the  equilibrium.  If  now  these  spino-cerebellar  tracts,  which 
occupy  the  margin  of  the  lateral  column,  are  completely  divided  in  the  dog, 
on  one  or  both  sides  hypotonia  results  in  the  extremities  on  the  same  side  and 
a  disturbance  in  regulation  of  the  principal  movements  in  the  roots  of  the 
extremities,  which  manifest  themselves  in  standing,  as  well  as  in  walking, 
as  abnormal  spreadings  and  overcrossings.  These  disturbances,  which 
are  suggested  already  in  total  division  of  the  dorsal  spino-cerebellar  tract, 
appear  in  full  severity  only  in  total  division  of  both  spino-cerebellar  tracts, 
the  dorsal  and  the  ventral.  But  all  these  pathological  phenomena  retro- 
gress quickly,  so  that  after  four  weeks,  even  with  bilateral  disjunction  of 
these  tracts,  scarcely  any  disturbance  is  present. 


234  DISEASES  OF  THE  SPINAL  CORD 

Quite  similar  phenomena  of  loss  of  function  of  similarly  transitory 
nature  appear  also  after  isolated  anterior  column  disjunction  in  the  upper 
cervical  portion  of  the  cord,  so  that  we  must  ascribe  also  to  the  anterior 
column  centripetal  conduction  for  the  deep  sensibility,  especially  for  the 
muscle  sense.  Especially  severe  is  the  appearance  of  these  disturbances  of 
the  muscle  sense  in  combined  disjunction  of  the  anterior  and  lateral  columns; 
on  the  other  hand,  the  intact  lateral  columns  can  almost  completely  take 
over  all  the  innervations  concerned  here,  after  total  disjunction  of  the  anterior 
and  posterior  columns,  followed  by  temporary  disturbances. 

It  is  probable,  therefore,  that  in  the  conduction  of  the  impulses  brought 
together  in  the  muscle  sense,  all  columns  of  the  spinal  cord  are  concerned, 
even  though  under  normal  conditions  the  main  conduction  may  be  a  func- 
tion of  the  lateral  columns.  The  larger  number  of  impulses  concerned  here 
ascend  in  the  same  half  of  the  spinal  cord;  but  for  the  position  sense,  for 
instance,  a  bilateral  path  of  conduction  seems  to  be  present. 

If  we  again  consider  the  conducting  paths  of  the  centripetal  impulses, 
especially  as  they  appear  after  the  experiments  on  the  dog,  we  must  say, 
that  the  posterior  columns  have  no  specific,  irreplaceable  function.  They  are 
important  factors  in  the  conduction  of  the  tactile  sensations,  and  are  prob- 
ably able  to  take  over  a  part,  if  only  a  small  one,  of  the  conduction  for  the 
muscle  sense.  The  anterior  columns  are  the  second  pathway  for  the  conduc- 
tion of  tactile  sensations,  are  feebly  active  in  the  conduction  for  the  pain 
sensation  the  fibres  probably  ending  subcortically,  and  take  a  not  incon- 
siderable part  in  the  conduction  of  the  deep  sensibility.  The  lateral  columns 
finally  are  the  main  tracts  for  the  pain  and  temperature  sense,  for  the  pres- 
sure sense  and  also  for  deep  sensibility;  only  for  the  delicate  tactile  sensations, 
they  possess  no  direct  components,  but  can  indirectly  destroy  them  by  very 
severe  disturbance  of  the  pressure  sense.  • 

Incidentally  the  curious  fact  must  be  mentioned  here,  that  the  posterior 
columns,  the  disjunction  of  which  does  not  at  all  interfere  with  pain  con- 
duction, represent  the  most  sensitive  place  (to  pain)  of  the  entire  spinal 
cord — perhaps  even  of  the  entire  central  nervous  system.  Even  the  thor- 
oughly anaesthetized  animal  starts  up  whimpering  when  the  columns  are 
pricked,  and  this  is  not  because  of  tearing  or  cutting  the  root,  which  in  fact 
is  not  nearly  so  painful,  but  there  must  be  special  endogenous  pain  tracts 
of  the  spinal  cord,  which  course  in  the  posterior  column. 

Complete  division  of  an  entire  half  of  the  spinal  cord,  in  both  dog  and 
monkey  is  followed  at  once  by  disturbances  of  the  movements  of  the  extrem- 
ities on  the  same  side,  which  in  the  monkey  are  more  serious  and  of  longer 
duration  than  in  the  dog,  but  which  in  both  animals  disappear  to  a  great 
extent  in  a  few  weeks,  corresponding  to  the  relations  of  the  motor  conducting 
tracts,  as  discussed  above.  So  far  as  disturbances  of  the  various  sensory 
qualities,  after  such  a  division  of  the  spinal  cord  are  concerned,  it  is  shown 


SENSORY  TRACTS  IN  MAN  235 

by  numerous  competent  experiments,  that  the  muscle  sense  is  disturbed 
much  more  on  the  side  of  the  division  and  that  the  pain  and  temperature, 
as  well  as  the  pressure  sense,  are  especially  affected  on  the  opposite  side. 
But  in  the  dog  none  of  these  forms  of  sensibility  are  destroyed  by  a  half 
sided  lesion  of  the  cord,  in  fact,  as  a  rule,  they  are  generally  restored  almost 
to  their  normal  condition  in  the  course  of  time.  Each  of  the  two  halves 
of  the  spinal  cord,  then,  is  able,  to  a  certain  degree,  to  conduct  sensory 
impulses  for  both  halves  of  the  body.  But  even  after  two  hemi-lesions  of 
the  spinal  cord,  which  at  a  distance  of  several  spinal  segments,  affect,'  now 
the  right,  now  the  left  half,  the  sensory  conductivity  is  not  abolished,  nor  is 
it  entirely  destroyed  by  a  third  lesion  a  few  segments  higher,  on  the  first  side. 

How  do  these  results  attained  in  the  higher  mammals,  compare  with 
those  reached  in  man?  Here  we  have,  it  is  true,  the  immeasurable  advan- 
tage, that  by  the  help  of  speech  much  more  exact  information  may  be  had 
about  the  sensations  brought  to  consciousness;  but,  on  the  other  hand,  it  is 
only  seldom  that  definite  focal  injuries  are  observed  with  such  clear  phenom- 
ena of  loss  of  function,  as  animal  experiments  offer  us.  Thus,  for  instance, 
in  the  posterior  column  affections  of  the  tabetics,  there  is  never  mere  loss  of 
the  posterior  column  fibres,  but  a  combination  with  diseases  of  other  pos- 
terior root  fibres,  which  end  in  the  gray  substance,  so  that  naturally  no 
conclusions  can  be  drawn  concerning  the  functions  of  the  posterior  column 
from  the  phenomena  of  functional  loss,  observed  in  such  cases.  Also  in 
tumors,  which  develop  in  definite  parts  of  the  spinal  cord,  for  instance  in  the 
lateral  column,  one  must  be  very  careful  in  interpretation  of  the  symptoms, 
because  of  their  distal  effects. 

The  best  observations  in  human  pathology  for  establishing  the  conducting 
tracts  of  sensation  in  the  spinal  cord,  therefore,  are  presented  by  the  punctured 
wounds  of  the  spinal  cord.  These  occur,  as  a  rule,  in  young  healthy  individ- 
uals, and  are  comparable,  in  the  simple  division  of  definite  portions  of  the 
spinal  cord,  with  the  conditions  of  the  animal  experiment.  Here,  however, 
there  is  an  insufficiency  of  autopsy  findings. 

Punctured  wounds,  in  which  the  knife  enters  the  vertebral  canal  from 
behind,  are  followed,  as  a  rule,  by  the  appearance  of  the  Brown-Sequard 
symptom-complex.  Motility  and  muscle  sense  are  lost  on  the  side  of  the 
injury,  pain  and  temperature  sense,  and,  also  to  a  greater  or  less  extent,  the 
pressure  and  tactile  sense  on  the  opposite  side.  On  the  injured  side  a  slight 
tactile  hyperesthesia  is  noticed;  also  as  a  rule  at  the  upper  boundary  of  the 
anaesthesia,  a  small  hyperaesthetic  zone  may  be  detected.  In  addition  to 
punctured  wounds  with  approximately  clean  division  of  half  the  spinal  cord, 
this  symptom  complex,  more  or  less  pronounced,  appears  in  cases  of  tumor, 
softening,  hemorrhage,  meningomyelitis  of  the  spinal  cord,  which  are  local- 
ized preeminently  in  one-half  of  the  spinal  cord  (Fig.  58) . 

We  have  already  said  above,  that  the  loss  of  motility,  appearing  after 


236 


DISEASES  OF  THE  SPINAL  CORD 


a  unilateral  lesion  of  the  spinal  cord,  is  capable  of  marked  retrogression, 
especially  in  the  lower  extremity.  So  far  as  the  individual  qualities  of 
sensation  are  concerned,  the  crossed  conduction  for  the  pain  sense  is  un- 
doubtedly far  more  developed  in  man  than  in  animals.  First,  in  all  com- 
plete divisions  of  one-half  the  spinal  cord  and  even  of  a  lateral  column,  the 
pain  sense  of  the  opposite  half  of  the  body  is  lost,  and  limited  restitution  is 


Fig.  58. — Left  hemisection  of  cord,     a,  Motor  and  vasomotor  paralysis,  b  and  d,  anaesthesia  of 
the  skin;  c,  hyperesthesia  of  the  skin.     {After  Erb.) 

observed  only  after  the  lapse  of  considerable  time.  But  there  are  a  long 
line  of  observations  on  record,  in  which  after  a  one-sided  lesion  of  the  spinal 
cord,  complete  loss  of  the  pain  sense  was  observed  to  continue  for  many 
years.  But  in  all  these  cases  the  lesion  seems  to  have  extended  to  the  other 
half  of  the  spinal  cord  also.  From  the  few  cases  that  lived  for  years  after  a 
punctured  wound  of  the  cord,  and  whose  spinal  cord  condition  could  then  be 


SEXSORY  TRACTS  IN   MAN  237 

ascertained,  it  may  be  concluded  with  certainty  that  in  man  also,  pain  sen- 
sations, may  be  conducted  though  very  incompletely  along  spinal  cord  tracts 
of  the  same  side.  Thus  appears  occasionally  the  symptom  of  allocheiria,  in 
which,  as  the  pain  stimulus  is  carried  only  to  the  centers  of  the  cerebral 
hemisphere  of  the  same  side  and  hence,  as  usual  projected  upon  the  oppo- 
site side  of  the  body,  the  pain  is  not  felt  at  the  point  of  injury,  but  at  the 
corresponding  place  in  the  other  half  of  the  body. 

The  temperature  sense  in  man  is  conducted  exclusively  and  normally,  in 
the  opposite  half  of  the  body,  and  as  a  rule  does  not  seem  to  be  restored,  even 
after  a  unilateral  lesion  of  the  spinal  cord  has  persisted  for  years.  But  we 
have  at  least  one  trustworthy  observation,  in  which  the  preservation  of  the 
lateral  column  on  the  same  side  sufficed  in  spite  of  destruction  of  the  entire 
opposite  half  of  the  spinal  cord,  to  make  an  incomplete  restitution  of  the 
heat  sense  possible  after  some  years.  Furthermore,  several  observations 
of  almost  isolated  destruction  of  the  antero-lateral  column  teach,  that  in 
man  also,  under  normal  conditions,  the  entire  conduction  for  the  pain  and 
the  temperature  sense  passes  through  this  column.  But  all  the  objections 
that  were  quoted  against  the  transference  of  this  conduction  to  Gower's 
tract  in  animal  experimentation,  must  be  renewed  here. 

A  separation  of  the  pressure  sense  from  the  more  delicate  tactile  sensa- 
tions, has  been  accurately  observed  in  only  a  few  instances  in  man.  At 
any  rate,  the  pressure  sense,  far  more  than  the  pain  and  temperature  senses, 
employs  in  addition  to  the  opposite  tracts,  a  less  important  system  of  con- 
duction on  the  same  side.  For  this  conduction  also,  the  antero-lateral  column 
seems  to  take  first  rank.  To  what  extent  posterior  and  anterior  columns 
may  serve  as  compensating  tracts,  has  not  yet  been  definitely  determined. 

For  tactile  sensations,  there  is  definite  proof,  from  exact  analysis  of 
competent  observations,  that  normally  it  must  employ  two  tracts,  one  on 
the  same,  the  other,  on  the  opposite  side.  In  many  cases  of  unilateral  lesions 
of  the  spinal  cord,  therefore,  the  tactile  sensations  are  retained,  while  the 
pain  and  the  temperature  senses  are  lost.  There  is  no  doubt  that  one  of  the 
tracts  for  the  tactile  sensations  courses  in  the  posterior  column  of  the  same 
side.  On  the  contrary,  the  frequently  assumed  hypothesis,  that  the  second 
tract  uses  the  opposite  lateral  column,  is  untenable  in  view  of  the  observa- 
tions presented;  this  second  crossed  tract  must  in  accordance  to  the  results 
of  the  animal  experiments,  pass  through  the  anterior  column.  Therefore, 
in  punctured  wounds,  in  which  the  knife  penetrates  obliquely  from  behind, 
and  in  which,  as  a  rule,  one  lateral  and  both  posterior  columns  are  destroyed, 
the  tactile  sensations  are  retained  on  both  sides,  since  both  anterior  columns 
remained  uninjured.  That,  on  the  other  hand,  the  long  posterior  column 
fibres,  that  pass  without  interruption  through  the  gray  matter  of  the  spinal 
cord  to  the  medulla  oblongata,  conduct  tactile  sensation  is  proven  by  the 
retention  of  the  latter  in  extensive  cavity  formations  in  the  gray  substance 


238  DISEASES  OF  THE  SPINAL  CORD 

of  the  spinal  cord,  though  the  pain  and  the  temperature  senses  are  lost 
(partial  sensory  paralysis). 

If  we  turn,  finally,  to  the  muscle  sense,  we  find  that  the  position  sense, 
about  which  alone  we  have  exact  knowledge,  is  at  first  lost  in  hemilesions 
of  the  cord,  on  the  side  of  the  lesion,  but  is,  as  a  rule,  considerably,  even 
though  not  entirely,  recovered.  Here  too,  in  man,  the  anterior  columns 
are  concerned  in  the  condition  as  well  as  the  spino-cerebellar  lateral  column 
tracts  while  the  degree  of  participation  of  the  posterior  column  is  as  yet 
doubtful. 

The  Brown-Sequard  symptom  complex,  is,  accordingly  in  man  only  in  so 
far  present  as  at  first  after  a  half  sided  lesion,  the  motility  and  the  muscle 
sense  are  lost  on  the  same  side,  the  pain  and  temperature  and  mostly  the 
pressure  senses  on  the  opposite  side,  whereas  the  tactile  sensations  from  the 
very  beginning  have  conduction  upon  both  sides.  But  neither  the  conduc- 
tion on  the  same  side  for  motility,  nor  that  on  the  opposite  side  for  sensibility, 
is  absolute.  An  extensive  restitution  of  the  functions  through  the  other 
half  of  the  spinal  cord  takes  place,  a  phenomenon  of  the  greatest  importance 
for  therapeutic  efforts  in  human  pathology. 

If  now  this  one  sided  lesion  of  the  spinal  cord  affects  the  upper  cervical 
portion,  another  series  of  phenomena  of  loss  of  function  may  be  observed  in 
addition.  First  respiration  ceases  on  the  side  of  the  lesion,  as  the  descend- 
ing tract  from  the  centers  of  the  medulla  oblongata  to  the  phrenicus-center 
in  the  fourth  cervical  segment  is  interrupted.  This  cessation  of  the  working 
of  half  of  the  diaphragm  is  permanent  in  man  as  well  as  in  the  dog  and  the 
ape.  Yet,  if,  in  the  animal  experiment,  after  a  duration  for  weeks  and 
months  of  the  inactivity  of  one  side  of  the  diaphragm,  the  phrenic  nerve  of 
the  other  half  of  the  diaphragm,  that  has  continued  its  functions,  be  cut,  and 
thereby  this  half  also  be  made  inactive,  then,  the  paralyzed  half  immedi- 
ately begins  to  work  again  (Porter's  experiment).  The  respiratory  stim- 
uli, descending  from  the  medulla  oblongata  in  the  sound  half  of  the  spinal 
cord,  and  which  are  now  no  longer  able  to  reach  the  same  half  of  the  dia- 
phragm, make  a  commissural  path,  at  the  level  of  the  fourth  cervical  seg- 
ment, to  the  phrenicus  center  on  the  other  side,  thus  awaking  it  to  renewed 
activity;  a  peculiarly  instructive  example  of  possible  processes  of  restitution 
in  the  central  nervous  system.  These  spinal  respiratory  paths  pass,  as  has 
been  proven,  by  attempts  to  sever  them  in  the  dog,  for  the  most  part  in  the 
ventral  part  of  the  antero-lateral  column,  to  a  lesser  degree  in  the  lateral  part 
of  the  anterior  column. 

Furthermore,  one  observes  in  both  animal  experiments  and  in  man, 
after  a  high  half  sided  cervical  lesion,  an  increase  in  cutaneous  temperature 
on  the  side  of  the  injury,  which  is  said  to  be  due  to  a  paralysis  of  vasomotor 
tracts  as  they  pass  from  the  medulla  oblongata  to  the  spinal  cord,  coursing 
more  than  likely,  also,  in  the  antero-lateral  column. 


TABES  DORSALIS  239 

Finally,  let  us  recall  once  more  the  oculo-pupillary  symptoms  after  a  half 
sided  lesion  of  the  cervical  portion  of  the  cord.  They  consist  of  narrowing 
of  the  palpebral  fissure,  of  contraction  of  the  pupil  and  occasionally  also,  of 
a  retraction  of  the  eyeball  (enophthalmos) ,  and  are  due  to  a  severing  of  the 
sympathetic  fibres,  passing  from  the  medulla  oblongata  to  the  oculo-pupil- 
lary center  in  the  first  dorsal  segment.  Here,  too,  a  definite  restitution  of 
the  nervous  conduction  is  apparently  possible,  through  the  other  half  of  the 
spinal  cord. 

Concerning  the  many  finer  details  of  diagnostic  importance,  especially 
with  regard  to  the  lowest  segment  of  the  spinal  cord,  we  must  refer  the  reader 
to  the  separate  chapters  on  the  pathology  of  the  spinal  cord. 

2.  SYSTEMIC  DISEASES  OF  THE  SPINAL  CORD 

(a)  Tabes  Dorsalis 

(Ataxie  locomotrice  progressive. — Locomotor  ataxia. — Ruckenmarkschwindsucht) 

BY 

S.  SCHOENBORN  (Heidelberg) 

We  designate  as  tabes  dorsalis,  or  tabes,  the  most  frequent  of  the  systemic 
degenerations  of  the  spinal  cord,  the  so-called  gray  degeneration  of  the 
posterior  columns  and  the  posterior  roots.  This  degeneration  in  general, 
must  be  considered  as  progressive  and  incurable.  It  offers  a  great  mass  of 
classic  clinical  symptoms,  among  which  those  of  the  motor  sphere,  with  the 
exception  of  ataxia,  occupy  a  very  insignificant  position.  On  the  other  hand, 
the  subjective  and  objective  disturbances  of  sensation — and,  indeed,  of 
almost  every  quality  thereof — those  of  the  reflexes,  of  the  sensory  and  trophic- 
functions,  play  the  main  role. 

The  (Etiology  of  tabes  is — at  least  in  the  way  the  question  is  usually  formu- 
lated— to  be  regarded  as  sufficiently  clear  to  us  to-day.  We  do  not  know 
the  "excitant  factor  of  tabes,"  if  there  is  such  a  one,  but  we  do  know  that 
the  disease  almost  always  appears  only  in  people  who  have  had  syphilis  at 
one  time.  In  the  great  majority  of  cases  it  is  a  meta-syphilitic  disease. 
This  states  the  main  fact  about  its  aetiology;  we  must,  however,  enter  in 
somewhat  more  detail  upon  the  theme,  that  has  been  the  subject  of  ardent 
dispute  for  decades. 

The  ideal  proof  of  the  connection  between  syphilis  and  tabes  would  be 
established,  if  we  could  only  succeed  in  proving,  the  same  excitant  clinically 
in  both  diseases.  We  may  consider,  to-day,  as  the  cause  of  syphilis,  the 
Schaudinn-Hoffmann  spirochaeta  pallida;  but  it  has  not  yet  been  possible  to 
find  in  the  cerebro-spinal  fluid  withdrawn  by  lumbar  puncture  or  in  the 
central  nervous  system  of  (pure)  tabetics,  spirochetes  (this,  of  course, 
because  of  the  nature  of  tabes  as  a  metasyphilitic  disease,  was  a  priori  to  be 


24o  DISEASES  OF  THE  SPINAL  CORD 

supposed  improbable).  [Very  recently  the  spirochsetes  have  rarely  been 
found.]  The  proof  of  the  presence  of  syphilitic  anti-bodies  in  tabetics 
(Wassermann-Plaut  reaction)  has  indeed  recently  been  successfully  shown 
with  comparative  constancy  (Citron),  but,  in  the  first  place,  there  is  as 
yet  but  little  clinical  material  and,  secondly,  we  must  lay  emphasis  on 
the  fact  that  the  reaction  (which  strikingly  appeared  rather  infrequently  in 
the  cerebro-spinal  fluid,  and  mostly  in  the  serum)  proved  that  the  tabetic 
very  probably  (even  when  he  denies  it)  has  at  some  time  had  syphilis,  but 
does  not  at  all  prove  that  tabes  depends  upon  syphilis,  since  most  non- 
tabetics,  who  had  been  infected  with  syphilis,  similarly  showed  anti-bodies. 
Besides  these  direct  methods,  pathological  anatomy  also  fails,  as  it,  as  we 
shall  see  below,  shows  the  lack  of  really  specific  changes  (in  so  far  as  the 
word  "specific"  may  be  used  here)  in  tabes. 

For  the  present,  therefore,  the  statistical  method  of  the  proof  of  the  exist- 
ence of  syphilis  remains  the  most  serviceable  for  our  purposes.  And  this 
teaches  clearly  and  indubitably  that 

i.  Almost  all  tabetics  have  at  one  time  had  syphilis,  whereby  of  course 
an  exact  anamnesis  (or  circumstantial  evidence  in  certain  cases)  must 
often  suffice  as  proof.  The  largest  existing  table  of  statistics,  collected  by 
Erb  (1904:  1 100  cases)  gives  about  90%  of  syphilitically  infected  tabetics; 
the  French  writers  (  Fournier,  Dejerine,  Belugon  and  Fame)  77  to  97%  of 
former  syphilitics,  Striimpell  90%,  Gowers  75  to  80%,  Negro  89%,  Homen 
83  %,  etc.  The  number  of  authors  crediting  only  a  small  percentage  to 
syphilis  (Storbeck  and  Gutmann),  grows  constantly  smaller  and  smaller,  and 
we  may  accept  the  fact  to-day  as  established,  that  approximately  90%  of  all 
tabetics  were  formerly  infected  with  syphilis.  All  "proofs  to  the  contrary" 
constructed  partly  at  least  artificially,  serve  in  objective  judgment  only  to 
confirm  these  results.  Of  10,000  non-tabetic  patients,  only  21.5%  had 
syphilis  in  their  anamnesis  (Erb).  The  fact  that  of  all  syphilitics  only 
comparatively  few  get  tabes,  proves  that  tabes  is  a  rare,  and  not  a  frequent 
sequela  of  syphilis. 

For  the  anamnestic  proof  of  syphilis,  it  is  of  course  essential,  that  the 
physician  knows  "how  to  ask  questions."  The  unpleasant  fact  of  a  past 
experience  with  syphilis  is  an  admission  that  nobody  cares  to  make;  very 
many  patients  (especially  of  the  lower  classes,  women,  etc.),  are  not  even 
acquainted  with  the  fact  of  having  had  syphilis  or  have  forgotten  it,  either 
because  it  was  but  slightly  treated  or  not  at  all  (this  appears  with  extra- 
ordinary frequency  in  the  case  of  those  who  become  tabetics  later  or  because 
its  course  was  brief).  Then  one  must  seek  for  indications:  eruptions,  swell- 
ing of  the  glands,  infections  of  members  of  the  family,  abortions  in  the  wife 
or  sterility,  relapsing  cerebral  paralyses,  etc.,  in  the  history,  leukoderma, 
plaques  in  the  mouth,  scars  of  all  sorts,  in  rare  cases  also  for  typical  tertiary 
diseases,  gummata,  etc.,  in  the  physical  examination.     In  tabes  of  children 


CAUSATION  OF  TABES  241 

(rare)  one  must  be  on  the  watch  for  extragenital  infection  or  for  congenital 
syphilis.  The  search  succeeds  more  easily  with  men  than  with  women,  and 
more  easily  in  the  upper  than  in  the  lower  classes.  Certain  occupations, 
which  seem  peculiarly  to  increase  this  liability  to  the  acquisition  of  syphilis, 
exhibit  the  most  tabetics  (merchants,  travellers,  actors,  officers),  others, 
correspondingly  the  fewest  (clergymen).  The  history  of  the  individual 
peculiarities  of  the  infection  {only  hard  chancres,  eruptions,  condylomata, 
etc.),  varies  considerably;  in  general,  it  seems,  as  has  been  remarked  above, 
that  the  majority  of  apparently  slight  and  but  little  treated  cases  lead  to  future 
tabes.  The  time  of  the  appearance  of  tabes  after  syphilis  is  also  somewhat 
variable,  but  it  appears  scarcely  ever  earlier  than  5  or  later  than  20  years  after 
the  infection;  in  late  infection,  also,  the  outbreak  of  the  spinal  cord  disease 
can  be  established,  occasionally,  only  close  to  old  age.  Tabetics  are,  more- 
over, nearly  always  immune  to  syphilis.  Tabetic  couples,  i.  e.,  when  husband 
and  wife,  one  after  the  other,  are  attacked  by  tabes,  are  syphilitic,  almost 
without  exception  {Fischler).  Why  in  certain  races  which  are  at  least  in 
part  fairly  saturated  with  syphilis,  tabes  appears  but  comparatively  seldom, 
has  not  yet  been  completely  explained  (cf.  below).  (Tabes  was  never  seen 
in  the  American  Negro  until  recent  years.  To-day  it,  and  paresis,  are 
becoming  common.     Burr.) 

2.  The  remaining  ^etiological  possibilities  worthy  of  any  consideration — 
compared  with  syphilis — are  of  but  slight  moment.  In  this  matter,  however, 
the  statistics  are  unfortunately  but  slightly  satisfying,  since  only  a  few  writers 
have  objectively  considered  all  such  possibilities  with  complete  impartiality. 
Erb  has  done  this  most  thoroughly  (cf.  below).  As  to  these  remaining 
factors,  it  appears  that  the  poisons  and  infections  that  are  worth  mentioning, 
outside  of  syphilis  (mercury,  alcohol,  tobacco,  influenza,  gonorrhoea)  with 
the  exception  of  gonorrhoea  alone  play  an  insignificant  role;  according  to 
Erb,  90%  of  the  tabetics  had  gonorrhoea;  but  for  the  most  part  they  were 
those,  who  at  some  time  also  had  suffered  from  syphilis.  Sexual  excesses,  in 
so  far  as  can  be  ascertained,  also  play  a  small  part;  on  the  other  hand, 
traumatism  may  give  rise  to  tabes  under  certain  conditions.  "  Catching  cold," 
on  the  contrary,  the  "rheumatism"  in  the  anamnesia,  is,  in  a  disease,  that 
begins  so  frequently  with  pulling  and  tearing  pains,  to  be  regarded  skeptically 
and  actually  can  be  but  seldom  demonstrated  on  closer  investigation.  The 
neuropathic  disposition,  the  "ectodermal  weakness  of  the  germinal  layer" 
{Bittorf)  doubtless  co-operates,  though  we  must  note,  that  genuine  signs  of 
physical  degeneration  in  the  patients  are,  in  our  experience,  of  less  frequent 
and  regular  occurrence,  than  nervousness  in  the  family.  Finally,  we  must 
mention  the  theory  of  tabes  as  the  "exhaustion  disease"  (Aufbrauchkrank- 
heit)  of  the  nervous  system  {Edinger) .  In  physical  overexertion,  particularly 
where  the  activity  of  the  individual  is  limited  to  one  side  of  the  body,  there 
takes  place  according  to  Edinger  an  early  using  up  of  the  nerve  substance 
16 


242  DISEASES  OF  THE  SPINAL  CORD 

which  may  develop  into  systemic  degeneration  of  the  tracts  concerned.  This 
holds  good,  incidentally,  for  nervous  regions,  weakened  by  other  kinds  of 
diseases,  poisons,  etc.,  and  thus  especially  for  the  spinal  cord  of  tabetics, 
which  has  been  influenced  by  syphilis.  The  hypothesis  is  quite  illuminating, 
and  holds  good  for  many  tabetic  symptoms  (thus,  for  instance,  after  extra- 
ordinary exertion  of  the  legs,  as  in  the  case  of  Marathon  runners,  it  happens 
not  infrequently,  that  the  patellar  reflexes  do  disappear,  but  they  remain 
absent  for  a  long  time  or  even  permanently,  only  in  those  individuals  who 
have  had  syphilis) ,  but  the  theory  does  not  explain,  why,  among  the  countless 
individuals  who  have  had  syphilis,  and  who  have  frequently  undergone  one- 
sided physical  over-exertion,  tabes  does  not  appear  far  more  frequently  than 
actually  happens. 

Only  Erb,  as  we  have  said,  has  made,  exact  investigations  of  the  condi- 
tions having  a  possible  aetiological  relation  to  tabes,  which  are  worthy  of  con- 
sideration and  has  put  them  together  in  tabulated  form.  It  appears,  natu- 
rally, that  very  frequently  several  aetiological  factors  co-operate;  but  if  one 
counts  only  the  cases,  in  which  only  one  aetiological  factor  was  proved  to  be 
possible  (with  the  high  percentage  of  syphilis  in  anamnesia,  this  number 
is  naturally  small)  one  finds  in  cases  of 

Syphilis  alone 27.0% 

Neuropathic  heredity  alone 0.7% 

Cold  alone i-4% 

Over-exertion  alone 0.3% 

Sexual  excesses  alone ■. 1 .0% 

Traumatism  alone 0.3% 

As  to  the  manner  of  the  working  of  all  these  evil  influences,  we  have 
unfortunately,  up  to  now,  only  conjectures;  the  majority,  it  is  probable, 
furnish  only  the  exciting  factor  for  the  development  of  the  disease.  But 
how  the  spinal  cord  becomes,  primarily,  less  resistant  or  more,  receptive  to 
the  exciting  stimulus,  we  do  not  know.  This  question  is  most  important 
as  to  syphilis:  Is  it  only  the  most  frequent  "agent  provocateur,"  or  the 
real  cause  of  tabes?  Many  observations  speak  for  the  latter  view.  I  must 
mention  also  the  hypothesis  of  French  authors  of  a  special  "nerve  syphilis," 
a  "syphilis  a  virus  nerveux,"  which  causes  but  slight  phenomena  to  appear 
on  the  skin,  mucous  membranes,  etc.,  outside  of  the  primary  chancre,  but 
lays  the  stress  of  its  dire  .work  to  the  destruction  of  the  nerve  elements, 
upon  which  is  based  the  future  tabes  or  paralysis.  Many  striking  phenom- 
ena, as  the  occurrence  of  tabes  in  married  couples,  the  frequency  of  cases 
of  tabes  among  syphilitics  whose  infection  came  from  the  same  source 
(Brosius)  might  thus  be  explained. 

Another,  at  first  sight  amazing  hypothesis  (Loewenthal) ,  assumes  that 
not  syphilis  itself  or  its  toxins,  but  the  protective  substances  (anti-bodies) 


THE  HISTOLOGICAL  ANATOMY  OF  TABES  243 

produced  years  after  the  infection,  cause  tabes.  This,  in  any  case,  ex- 
tremely doubtful  hypothesis  is  to  be  merely  mentioned  here;  it  proves  only, 
as  does  all  that  has  been  said  above,  that  though  we  know  that  syphilis  is  the 
main  factor  in  the  (Etiology  of  tabes,  we  do  not  know  the  manner  of  its 
working. 

If  we  turn  to  the  pathological  anatomy  of  tabes,  things  are  simple  enough 
so  far  as  regards  histology,  more  complicated  as  to  the  distribution  of  the 
process  in  the  central  nervous  system,  and  extraordinarily  complicated  and 
difficult  to  explain  so  far  as  pathogenesis  is  concerned. 

If  we  summarize  our  present  knowledge  on  these  points,  we  may  say: 

Tabes  is  a  degenerative  disease,  characterized  by  progressive  destruction  of  the 
nerve  fibres  and  loss  of  the  medullated  sheaths  as  well  as  by  a  secondary  increase  of 
the  glia — -so-called  gray  degeneration.  It  is  located  in  the  posterior  roots  and 
the  posterior  columns — -first  in  the  column  of  Burdach,  then  in  GolVs  column — and 
in  a  few  less  constant  points.  It  begins  probably  with  a  specific  syphilitic  inflam- 
mation {in  the  broadest  sense)  in  the  posterior  roots  and  a  chronic  slight  menin- 
gitis, which — segment  after  segment  being  affected — gradually  causes  the  tracts 
of  the  central  organ  co-ncerned  to  degenerate.  We  shall  try7  to  prove  these 
statements  in  the  following. 

The  histological  process  may  almost  be  described  as  banal,  so  typically 
does  it  repeat  itself  in  the  majority  of  cases.  "The  nerve  fibres  degenerate, 
lose  their  medullated  sheaths  in  a  more  or  less  irregular  way,  are  in  part 
thickened  and  swollen,  later  atrophy,  together  with  the  axis  cylinders,  and 
finally  disappear  altogether.  The  proliferated  glia  appears  as  a  delicate 
fibrous  network,  in  which,  frequently,  the  gaps  left  by  the  destruction  of  the 
nerve  fibres  are  still  visible;  later  it  becomes  transformed  into  a  delicately 
waved,  striped,  tough  mesh,  in  which  nuclei  and  neuroglia  cells,  as  well  as 
corpora  amylacea  are  present  in  great  numbers.  The  vessels  are  but  slightly 
changed;  have  thickened  and  hyaline  walls,  narrowed  lumen  and  here  and 
there  a  somewhat  richer  intercalation  of  nuclei.  In  quickly  progressing 
recent  cases  granular  cells  have  also  been  found  (Erb)." 

Changes  quite  analogous  to  these  are  found  in  the  posterior  roots,  and 
especially  in  the  part  that  lies  between  the  entrance  into  the  dura  and  the 
spinal  ganglion,  which  French  writers  call  the  "nerf  radiculaire, "  since 
Nageotte  wrote  about  it,  and  here  and  there,  but  not  constantly  in  the  spinal 
ganglia  themselves.  This  division  of  the  roots  shows  interstitial  neuritis, 
and  later,  degeneration;  according  to  the  most  recent  investigations,  there 
appear,  in  the  beginning,  regenerative  processes  also  (Nageotte,  Marinesco), 
especially  in  the  form  of  "collateral  regeneration, "  but  the  process  always 
ends  in  total  destruction  of  the  fibres.  Finally,  the  meninges  are  frequently 
also — constantly  according  to  French  writers — affected  by  diffuse  prolifera- 
tion of  the  cells,  especially  in  the  walls  of  the  veins  and  the  arteries  of  the 
meninges,  which,  according  to  Nageotte  and  others,  is  identical  with  the 


244  DISEASES  OF  TEE  SPINAL  CORD 

changes  in  the  vicinity  of  the  vessels,  observed  in  genuine  syphilitic  processes 
(these  cells  are  also  found  in  large  quantities  in  the  cerebro-spinal  fluid  of 
tabetics) .  The  slight  meningitis — in  contradistinction  to  which  there  exists  a 
rarer  coarse,  chronic  meningitis,  with  thickening  and  adhesions— occurs  in  the 
vicinity  of  the  posterior  columns  and  the  points  of  entry7  of  the  posterior  roots. 
It  is  seldom  that  the  anterior  roots  show  a  similar  degeneration;  on  the  other 
hand,  we  find  frequently,  in  addition  to  the  changes  in  the  fibres,  degeneration 
in  the  ganglion  cells,  and  especially  in  those  of  Clarke's  columns,  rarely,  also 
in  those  of  the  anterior  horns  (the  latter  only  in  isolated  forms  of  tabes  with 
amyotrophy,  arranged  in  nests  or  foci,  Lapinsky).  Occasionally,  also, 
the  degeneration  affects  peripheral  motor  and  sensory  nerves,  but  almost  only 
in  the  cases,  in  which,  clinically  also,  the  simultaneous  presence  of  neuritis, 
on  account  of  existing  pareses,  etc.,  may  be  recognized. 

It  must  be  emphasized,  however,  that  the  changes  in  the  "nerf  radicu- 
laire"  and  the  meninges,  in  the  form  described,  are  an  outcome  of  French 
investigations  of  recent  years,  and  are  still  awaiting  full  confirmation;  but 
they  have  been  found  with  such  constancy  by  these  investigators,  that, 
considering  their  importance  for  pathogenesis,  we  are  justified  in  regarding 
them  as  peculiarly  significant. 

Macroscopically  a  tabetic  spinal  cord  can  often  be  easily  recognized 
by  the  small  volume,  the  "disappearance"  of  the  posterior  columns;  further- 
more the  portion  of  the  posterior  columns  affected  is  characterized  by  the 
easily  recognized  gray  discoloration,  not  only  when  the  cord  is  intact  but 
more  particularly  on  cross-section. 

These  are  the  real  tabetic  changes.  Besides  these,  we  find,  not  very 
rarely  pathological  pictures  which  belong  to  other  diseases  of  the  central 
nervous  system:  combined  diseases  of  the  columns  (with  participation  of 
the  lateral  cerebellar  tracts,  the  pyramidal  tracts,  etc.,  cf.  below),  pro- 
liferations in  the  molecular  layer  of  the  cerebellum,  formation  of  cavities 
(combination  with  syringomyelia),  and  genuine  gummatous,  syphilitic 
processes  of  the  meninges,  of  the  substance  of  the  brain  and  spinal  cord; 
finally  the  frequent  combination  with  progressive  paralysis,  even  from  an 
anatomic  standpoint. 

How,  then,  does  the  tabetic  process  spread? 

In  part  the  localization  has  been  already  mentioned:  the  membranes, 
posterior  roots,  posterior  columns.  In  detail,  we  can  start  from  the  degen- 
eration of  the  posterior  roots,  even  when  they  do  not  show  the  earliest  and 
most  marked  appearance  of  the  process,  as  occasionally  happens.  But  if 
we  assume  that  the  process  starts  regularly  from  the  posterior  roots  (cf. 
below),  we  should  have  to  see  the  degeneration  of  the  fibres  of  the  posterior 
roots  pass  over  into  the  posterior  columns,  and,  according  to  the  level  of  the 
spinal  cord,  affecting  more  either  the  columns  of  Burdach  or  those  of  Goll; 
it  would  have  to  rise,  in  part,  to  the  nuclei  of  the  posterior  columns  in  the 


MORBID  ANATOMY  OF  TABES  245 

medulla  oblongata,  and,  for  the  rest,  pass  over  into  Clarke's  columns  of  the 
posterior  horn  and  the  Lissauer  marginal  zone.  Also  the  descending  fibres 
in  the  posterior  column  would  have  to  undergo  degeneration.  Then, 
surely,  the  process  could  often  be  traced  up  to  the  spinal  ganglion  on  one 
side,  up  to  the  cortex  of  the  cerebellum,  into  the  inner  capsule  and  to  the 
sensory  centers  of  the  cerebral  cortex. 

This  actually  corresponds  to  the  course  of  the  disease  in  the  majority 
of  typical  cases,  with  the  one  restriction  mentioned,  that  the  first 
clearly  visible  changes,  appear,  as  a  rule,  not  in  the  posterior  roots, 
but  in  the  posterior  columns.  In  fact  the  first  change  observable  in  the 
lumbar  segment  is  a  symmetric  field  of  degeneration  in  the  columns  of 
Burdach,  laterally  next  to  the  posterior  horn,  the  very  place  of  the  "bande- 
lettes  externes,"  the  entry  zone  of  the  roots.  This  is  the  typical  condition 
at  the  initial  stage  of  the  disease.  Thence  the  process  continues  upwards. 
Corresponding  to  the  course  of  the  columns  of  Burdach,  with  the  degene- 
ration of  every  higher  segment,  the  disease  moves  more  towards  the  midline, 
finally  into  the  columns  of  Goll.  The  higher  we  go  in  the  spinal  cord, 
the  more  clearly  are  the  columns  of  Goll  affected  (in  regular  sequential 
succumbing  of  the  individual  segments),  so  that  we  can  lay  down  this 
rule:  in  the  lumbar  segment,  first  the  columns  of  Burdach,  then  the 
total  cross-section  of  the  posterior  columns  are  attacked;  in  the  dorsal  and 
cervical  segments,  first  the  columns  of  Goll,  only  later  the  entire  cross- 
section;  in  the  medulla,  the  nuclei  of  the  posterior  columns  in  the  vicinity 
of  the  calamus  scriptorius.  As  a  rule,  the  Lissauer  marginal  zone  and 
Clarke's  columns  of  the  posterior  horns,  are  affected  at  the  same  time,  the 
latter,  occasionally,  in  such  a  way  that  the  cells  remain  intact,  whereas  the 
fibres  degenerate.  The  posterior  roots  themselves  are  also  always  affected, 
even  if  not  always  apparently  to  the  same  extent,  as  the  posterior  columns, 
so  that  many  authors  assume  an  "elective  disturbance"  of  the  root  fibres. 
Very  often  we  find  cell  degeneration  in  the  spinal  ganglia,  even  if,  and  this 
must  be  emphasized,  not  absolutely  constant.  On  the  other  hand,  in  the 
lumbar  segment,  the  dorso-medial  bundle,  and  farther  up,  the  so-called 
ventral  posterior  column  field,  are  almost  invariably  spared. 

Concerning  the  participation  of  the  meninges,  which  may  be  described 
as  rather  constant,  we  have  spoken  above  under  histology.  Frequently 
we  find  also  affected  the  reflex  collaterals,  radiating  into  the  anterior  horn, 
in  the  rare  cases  of  combination  with  spinal  muscular  atrophy,  also  the 
ganglion  cells  in  the  anterior  horn.  Not  rarely,  the  gray  degeneration 
attacks  also  Gower's  bundle  and  the  lateral  cerebellar  tracts,  but  seldom, 
on  the  other  hand,  does  it  affect  the  pyramidal  tracts.  In  the  cortex  of  the 
cerebrum  and  of  the  cerebellum  we  occasionally  find  similarly  degenerated 
fibres.  The  peripheral  sensory  fibres  (rarely  the  motor  ones)  show,  at  times, 
a  degenerative  process,  which  can  not,  however,  compare  in  severity  with 


246  DISEASES  OF  THE  SPINAL  CORD 

the  columnar  degeneration.  Very  much  more  frequently,  however,  are  the 
cerebral  nerves  attacked,  and  especially,  and  almost  regularly,  the  opticus, 
in  the  sense  of  a  primary  parenchymatous  degeneration,  possibly  omitting  a 
small  bundle  (Marie  and  Z,m),  and  this  degeneration  seems  to  be  quanti- 
tatively independent  of  the  simultaneously  occurring  disappearance  of 
ganglion  cells  in  the  retina.  Next  in  frequency  the  abducens,  the  oculomotor 
and  trochlear  nerves  are  affected,  and,  far  more  rarely,  the  auditory,  olfac- 
tory, trigeminal.  Positive  findings  in  the  sympathetic  have  been  strikingly 
rare  (cf.  below). 

In  contradistinction  to  these  more  or  less  regular  systemic  degenerations 
in  the  normal  course  of  tabes  stand  some  apparently  irregular  cases,  in  which, 
for  instance,  there  are  scarcely  any  changes  in  the  lumbar  segments,  but  seri- 
ous ones  in  the  posterior  columns  of  the  cervical  region  (for  instance,  in  so- 
called  tabes  superior,  only  in  the  columns  of  Burdach  of  the  cervical  segments) , 
or  where  spots  of  degeneration  are  scattered  arbitrarily,  as  it  seems,  over 
the  posterior  columns.  If  we  follow  up  these  cases,  we  find  almost  invariably 
that  the  cause  is  an  irregular  sporadic  lesion  of  individual  posterior  roots, 
individual  segments,  which  can  even,  apparently,  be  confined  to  one  segment 
only.  From  all  this  it  appears  with  considerable  definiteness,  that  in  tabes 
we  have  a  segmentary,  progressive,  normally  ascending  disease  of  the  posterior 
root  regions,  that  is,  a  lesion  of  the  posterior  columns  of  a  radicular  type  (Erb) . 
Upon  this  pathologic-anatomic  fact,  Nageotte  and  other  French  authors 
base  what  is,  at  present,  the  most  plausible  theory  of  the  pathogenesis  of  tabes, 
into  which  we  shall  enter  briefly. 

According  to^Nageotte,  the  process  is  as  follows :  At  first,  there  develops 
a  diffuse  leptomeningitis,  which,  because  of  the  small-celled  infiltration  of  the 
pia  and  the  changes  in  the  vessels,  which  are  supposed  to  strongly  resemble 
syphilitic  changes,  he  regards  as  specific-syphilitic.  Serious  local  changes, 
adhesions,  etc.,  do  not  arise  from  this  (cf.  above);  on  the  other  hand,  there 
soon  occurs  a  root  neuritis  (cf .  above,  a  neuritis  of  the  part  between  the  dura 
and  the  spinal  ganglion).  Hence  the  degeneration  occasionally  extends  to 
the  spinal  ganglion,  but  always  the  entry  zone  of  the  roots  and  the  posterior 
columns,  and  is  even,  at  times,  most  clearly  visible  in  the  posterior  columns, 
since,  according  to  Nageotte,  the  collaterals  or  the  so-called  short  root  fibres 
may  degenerate  first  at  the  distal  end.  That  from  the  meninges,  the  nerf 
radiculaire  is  affected  with  special  ease,  is  explained  by  the  indubitable 
fact,  that  lymphatic  channels  pass  constantly  along  the  posterior  roots,  which 
can  bring  the  supply  of  syphilitic  and  other  poisons  to  bear  with  peculiar 
intensity  upon  the  restricted  space. 

This  theory,  doubtlessly,  meets  most  of  the  demands.  It  has  only  two 
weak  points.  One,  that  the  syphilitic  nature  of  the  meningitis  has  thus  far 
not  been  established,  since  we  simply  do  not  know  any  changes,  which  must 
be  regarded  as  certainly  syphilitic  and  which  can  be  proven  such  experiment- 


PATHOGENESIS  OF  TABES.     SYMPTOMS  247 

ally;  second,  that  up  to  the  present  time,  there  have  been  too  few  special 
anatomic  investigations  of  the  nerf  radiculaire. 

The  assumption  of  a  primary  disease  of  the  spinal  ganglia  {Marie  and 
others)  is  weakened  by  the  fact  that  the  changes  demonstrated  are  too 
inconstant;  nor  is  the  exceedingly  different  participation  of  the  incoming  and 
outgoing  nerves  favorable  to  the  theory. 

Likewise  the  assumption  of  a  diffuse  toxic  influence  acting  on  the  poste- 
rior columns  through  the  poison  circulating  in  their  lymphatic  channels, 
coming  from  a  meningitis  "posterior"  luetica  {Marie  and  Guillain)  is  a 
mere  hypothesis. 

Ley  den  and  Goldscheider  believe  the  process  to  have  its  beginning  in  the 
peripheral  sensory  nerves,  but  they  are  too  rarely  found  affected  to  make 
this  supposition  probable. 

The  possibility  that  a  primary  toxic  disease  of  the  intramedullary  root 
tracts  exists  at  the  outset,  which  is  the  theory  of  Striimpell  and  others,  is,  on 
the  other  hand,  not  to  be  set  aside  lightly. 

To  what  extent  the  results  of  the  investigation  of  the  cerebrospinal  fluid 
have  been  favorable  to  the  one  or  the  other  hypothesis,  we  shall  see  below. 

Now  the  question  arises;  are  these  pathologic-anatomic  processes,  which 
have  been  described,  of  syphilitic  origin  or  not?  It  is  impossible  for  us,  at  this 
time,  to  answer  either  in  the  affirmative  or  the  negative.  The  reason  for  this 
is,  that  the  means  for  certain  diagnosis  of  a  specific  syphilitic  change,  is  as  yet 
denied  us,  so  that  it  has  been  possible,  in  recent  times,  to  doubt  even  the 
specific  character  of  the  genuine  gummatous  changes.  So  long  as  we  can  not 
isolate  the  excitant  of  syphilis,  or  its  toxins  from  the  regions  affected,  or 
experimentally  evoke  a  typical  tabes,  by  the  use  of  syphilitic  matter,  we  shall 
have  no  positive  proof.  To-day  we  can  only  say,  that,  according  to  the  status 
of  our  present  knowledge,  it  is  possible,  but  that  it  has  thus  far  not  been 
proven,  that  the  changes  of  the  nervous  system  found  in  tabes  are  with  regard 
to  the  pathological  anatomy  of  a  specific  syphilitic  character.  But  the 
observations,  attesting  the  correctness  of  this  supposition,  are  multiplying. 
The  discussion  of  the  specific  tabetic  anatomic  changes  in  the  rest  of  the  body 
(arthropathies,  etc.),  will  follow  their  mention  under  symptomatology. 

The  clinical  symptoms  of  tabes  are  unusually  varied.  Their  distribu- 
tion into  stages  of  the  disease  (initial  or  preataxic  stage,  ataxic  stage,  para- 
lytic or  final  stage)  is  correct  only  in  isolated  cases,  because  of  the  differences 
in  the  course  of  the  disease,  and  is  subject  to  the  point  of  view  of  the  observer. 
Most  easy  to  demarcate  is  the  initial  stage,  the  duration  of  which,  however, 
may  vary  from  a  few  months,  or  even  weeks  to  22  years  (mentioned  by  Erb). 
But  it  is  characterized  by  symptoms  which  belong  regularly  to  this  stage. 

Since  it  is  usually  only  by  chance  that  the  diagnosis  of  tabes  is  made  in 
this  early  stage,  the  possibility  of  its  demarcation  depends  often  upon  the 
statements  of  the  patient,  who  has  already  reached  the  later  "  recognizable ' ' 


248  DISEASES  OF  TEE  SPINAL  CORD 

stages  of  the  disease.  The  most  constant  complaint  of  these  patients  to 
the  physician  is,  " I  have  had  rheumatic  pains  for  so  many  years"  (also  often, 
alas,  in  the  form  "my  doctor  has  been  treating  me  for  years  for  "rheu- 
matism"). This  "rheumatism"  is  with  tabetics  the  almost  invariable, decep- 
tive designation  of  the  lancinating  pains,  which  as  a  matter  of  fact  are  most 
commonly  the  first  symptom  of  the  disease.  Almost  all  tabetics  give  the 
same  description  of  this  disturbance:  lightning-like  shooting  pains,  lasting 
from  an  instant  to  several  seconds — very  seldom  of  longer  duration — which 
are  unusually  violent,  so  that  the  patients  often  scream  aloud.  As  a  rule, 
these  pains  do  not  appear  regularly  distributed  over  long  periods  of  time, 
but  rather  massed,  after  exertions,  excesses,  psychic  irritations,  exposure  to 
cold,  change  in  the  weather,  occasionally,  too,  without  any  apparent  cause. 
They  usually  follow  the  single  nerve  trunks  and  the  localization  of  the  disease 
in  the  spinal  cord  and  are  usually,  therefore,  observed  first  in  the  legs 
(sciatic,  crural),  only  later  in  the  arms  and  the  trunk,  very  seldom  in  the 
head.  By  no  means,  however,  do  the  pains  always  recur  in  the  same  nerve, 
but  occur  within  the  same  exacerbation  period  in  all  possible  regions  attacked; 
but  the  contrary  also  takes  place  at  times,  and  in  these  latter  cases  there  is 
often  hyperesthesia  to  touch,  pressure,  etc.,  in  the  regions  concerned.  The 
lancinating  pains  are  present  in  90%  of  all  tabetics  (Erb)  and  not  infrequently 
characterize  the  entire  picture.  There  are  patients,  who,  through  these 
terrible  pains  alone— having  very  few  other  symptoms — are  completely 
deprived  of  their  ability  to  work  and  their  joy  in  life  and  finally  commit 
suicide.  Very  many  more,  even  when  these  pains  finally  disappear  after 
many  years,  have  in  the  meantime  become  morphine  victims.  It  is  remark- 
able that  besides  the  crises  (cf.  below)  and  these  peculiar  lightning-like 
pains,  other  pain  is  not  frequent  in  tabes. 

Paresthesias,  the  subjective  sensory  disturbances,  are  considered  as  the 
next  early  symptom.  Many  tabetics  complain  of  their  feet  "falling  asleep," 
"feeling  prickly,"  of  a  sensation  of  "ants  crawling  over  their  feet,"  and, 
moreover,  this  disturbance  (not  taking  into  consideration  the  rare  cases  of 
tabes  superior,  cf.  above)  is  frequently  a  typically  ascending  one.  It  begins 
in  the  tips  of  the  toes,  separate  toes  become  "dead,"  then  the  soles  become 
paresthetic,  or  the  one  margin  of  the  foot,  or  the  entire  foot.  The  feet  are 
continually  cold  or  warm.  Then  the  paresthesias  become  stocking-like, 
the  feeling  of  a  band  about  the  knee  arises,  which  develops  later  and  higher, 
as  the  "  girdle  feeling"  in  the  trunk,  to  one  of  the  classic  tabetic  paresthesias, 
which  often  appears  as  an  early  symptom.  The  ascending  type,  which 
appears  also  in  myelitis,  is  occasionally  lacking;  the  patients  complain  of 
their  forearms  going  to  sleep  (ulnaris  sensation),  of  the  same  feeling  in  the 
rectum,  scrotum,  etc.  A  feeling  of  heaviness  in  the  legs  is  to  be  interpreted 
partly  as  a  pure  paresthesia,  partly  as  a  genuine  diminution  of  muscular 
strength.     This  also  is  very  frequent  in  the  initial  stage  as  subjective  dis- 


SYMPTOMATOLOGY  OF  TABES  249 

turbance.  The  patients  do  not  like  to  take  long  walks;  they  tire  quickly. 
At  this  time  also  there  usually  appear  disturbances  in  the  sphincters  and  of 
the  sexual  function.  In  the  beginning,  disturbances  of  the  bowels,  because 
of  the  great  frequency  of  bowel  sluggishness,  are  rarely  of  much  value 
{incontinentia  alvi  can  scarcely  occur  as  an  early  symptom);  on  the  other 
hand,  one  often  hears  that  the  patients  have  to  urinate  more  frequently, 
that  they  can  not  properly  hold  their  urine,  or  (more  seldom)  that  they  have 
to  exert  strong  pressure  in  urination  (incontinentia  vel  retentio  urinse). 
Male  patients  complain  almost  invariably  (80%),  and  usually  even  in  the 
early  stage,  of  impotence  in  the  widest  sense;  as  a  rule,  at  first,  of  insufficient 
erections,  premature  or  insufficient  ejaculation;  the  loss  of  the  libido,  to  the 
torture  of  the  patient,  usually  follows  only  later.  In  women,  similar  changes 
seem  to  take  place.  Frequently  the  stage  of  impotence  is  preceded  by  one  of 
increased  libido,  occasionally,  too,  of  increased  sexual  capacity. 

As  the  last  preataxic  symptom  of  a  subjective  nature,  we  note  disturbances 
affecting  the  eyes;  most  frequent  is  diplopia  which  often  appears  first  as 
"seeing  dimly,"  as  a  confused  picture,  etc.  Occasionally  it  persists  as  a 
constant  symptom,  but  it  usually  disappears,  yielding  to  other  phenomena; 
it  frequently,  however,  returns.  Other  patients  complain  first  of  a  decrease 
in  the  vision,  which  affects  usually  one  eye  at  the  start,  later  becomes  bi- 
lateral, and  may  lead  to  complete  amaurosis.  There  are  tabetics,  who  have 
absolutely  no  symptoms,  as  long  as  they  live,  except  total  blindness  and 
possibly  disturbances  in  the  tendon  reflexes. 

For  these  ocular  phenomena,  we  find  even  in  the  early  stage,  objective 
foundations;  disturbances  in  the  muscles  of  the  eyes  and  in  the  opticus,  as 
well  as  pupillary  rigidity  are  present.  Corresponding  to  the  diplopia  just 
mentioned  pareses  of  the  abducens  (most  frequent) ,  oculomotor  (ptosis  also 
very  frequent)  and  trochlear  are  often  temporary  in  the  beginning,  their 
intensity  changing  in  the  course  of  weeks,  even  of  days;  they  may  disappear 
completely  and  never  return.  But  often  in  the  later  stages  there  develop 
permanent,  usually  total,  paralyses;  we  find  ophthalmoplegia  interna  (paral- 
ysis of  all  the  inner  muscles  of  the  eye),  externa  and  total  ophthalmoplegia, 
in  which,  back  of  the  sunken  lid,  the  ball  usually  slightly  turned  inwards 
and  downwards  is  fixed  immovably  (Fig.  59). 

Changes  in  the  optic  nerve  are  frequent  in  the  ophthalmoscopic  picture, 
but  the  findings  are  fortunately  not  always  parallel  to  the  actual  visual 
disturbance.  The  disc  is  more  or  less  grayish-white,  in  later  stages  often 
discolored  to  a  porcelain  white,  thus  marking  the  presence  of  atrophy  of  the 
optic  nerve;  the  vessels  of  the  disc  are  contracted,  its  margin  marked  with 
unusual  sharpness;  the  retina  is,  as  a  rule,  unchanged.  In  cases,  in  which 
the  discoloration  from  gray  to  white  proceeds  very  rapidly,  there  appears  also 
a  progressive  loss  of  vision,  finally  leading  to  blindness;  on  the  other  hand, 
however,  one  sometimes  finds  in  tabetics,  who  complain  only  of  slight  visual 


250 


DISEASES  OF  THE  SPINAL  CORD 


disturbances,  pure  white  discs  in  the  final  stage.  Characteristic  is  the  in- 
creasing contraction  of  the  visual  field,  especially  for  colors,  which  may  lead 
to  a  partial  or  total  color  blindness  long  before  the  appearance  of  total 
amaurosis. 

By  far  the  most  frequent  of  all  changes  in  the  eyes,  however,  is  pupillary 
rigidity,  the  Argyll-Robertson  phenomenon;  it  appears  in  almost  all  instances 
even  in  the  early  stage  and  is  associated  with  sluggishness  of  reflexes. 
Each  eye  is  tested  separately  by  allowing  daylight  or  concentrated  light  to 
fall  on  the  previously  covered  eye;  the  pupil  contracts  slowly,  sometimes 
irregularly,  often  not  at  all.     Next  we  allow  the  patient  to  converge,  or  to 


Fig.  59. — Total  paralysis  of  the  oculomotor  on  both  sides.     Attempt  to  raise  the  lids 
(to  knit  the  brows)  prevented  by  ptosis. 

accommodate  for  distance;  in  both  cases  a  distinct  contraction  is  visible. 
Therefore,  there  is  pupillary  rigidity  to  the  stimulus  of  light,  whereas  the 
convergence  and  accommodation  reactions  are  retained.  This  is  the  rule, 
occasionally  even  the  only  early  symptom  in  tabes.  As  a  rule  the  pupils  are 
usually  of  unequal  width  in  the  beginning  (anisocoria) ,  one  usually  partly 
dilated  or  mydriatic,  the  other  in  many  cases  and  at  a  very  early  date  con- 
siderably narrowed  (to  the  size  of  a  pinhead) ;  not  seldom  they  are  no  longer 
round,  but  elliptic  or  angular  (without  the  presence  of  posterior  synechia). 
Also  the  dilatation  reflex  of  the  pupil  by  means  of  sensory  stimuli  (pricking 
the  skin  of  the  cheek),  is  absent  usually  in  advanced  cases. 


SYMPTOMATOLOGY  OF  TABES  251 

We  mentioned  above  the  subjective  sensory  disturbances  of  the  early  stage. 
One  of  them  is,  as  a  rule,  even  now  the  expression  of  an  objective  disturbance : 
the  insufficient  feeling  of  the  patients  relative  to  what  is  beneath  their  feet. 
They  do  not  know  whether  they  walk  on  carpets  or  boards,  on  clay  or  stone; 
they  have  the  feeling  of  "walking  on  velvet,"  etc.  Then  we  find  mostly 
a  dulling  of  tactile  sensations,  probably  also  of  the  pressure  sense,  whether  it 
be  in  the  plantar  surface,  in  the  outer  or  inner  margin  of  the  foot,  or  in  the 
toes.  Also  hypalgesia  and  analgesia  occur,  and,  likewise,  we  find  early  a 
slight  degree  of  hyperesthesia  to  cold  in  the  trunk,  which  may  be  subjec- 
tively represented  by  the  statement,  that  washing  with  cold  water,  to  which 
the  patient  has  been  formerly  accustomed,  has  become  unpleasant  or 
even  painful. 

As  the  last  of  the  actual  early  symptoms,  I  mention  the  changes  in  the 
tendon  reflexes.  Only  very  seldom  do  we  miss  in  the  initial  stage  a  weakening 
or  absence  of  one  or  more  tendon  reflexes.  Of  course,  to  establish  this,  it  is 
invariably  necessary  to  carefully  test  all  the  more  important  tendon  reflexes 
of  the  body,  above  all — after  the  patellar  reflex — the  Achilles  tendon  and 
the  triceps  reflexes.  Absolute  relaxation  of  the  muscles  and  joints  con- 
cerned in  the  test  is  the  necessary  condition,  before  one  can  speak  of  a 
reflex  being  absent  (for  the  methods  of  testing  the  reflexes  cf.  under  "gen- 
eral diagnostics");  at  every  such  examination  the  Jendrassik  or  other 
similarly  valuable  method  for  the  purpose  of  facilitating  and  increasing 
the  relaxation  must  be  employed.  If  these  precautionary  measures  are 
employed  the  earliest  symptom  in  tabes  is  found  to  be  a  difference  between 
two  symmetric  tendon  reflexes,  the  one  being  usually  normal  (scarcely 
ever  exaggerated),  the  other  diminished.  And,  moreover,  this  diminution, 
which  quickly  increases  to  a  "total  absence"  of  the  reflex,  by  no  means 
always  affects  the  patellar  reflexes  first  (Westphal's  phenomenon),  but  very 
often  the  Achilles  tendon  reflex,  or  in  tabes  superior  the  triceps  reflex. 

If  we  mention,  furthermore,  the  occasional  appearance  in  the  beginning 
stage  of  the  Romberg  phenomenon,  to  be  discussed  at  greater  length  below, 
we  shall  have  mentioned  in  a  nutshell  the  usual  early  symptoms  of  tabes,  to 
which  other  authors  (among  them,  Erb)  it  is  true  append  a  long  list  of  others. 
Meanwhile,  the  borderline  is  always  more  or  less  arbitrary,  unless  importance 
is  attached  to  the  appearance  of  ataxia,  which  is  often  absent  at  this  early 
stage.  The  initial  stage  often  passes  unnoticed  into  the  fully  developed 
disease,  which  we  must  now  discuss,  connecting  the  ataxic  with  the  so-called 
final  stage. 

As  mentioned  above  the  pains  may  accompany  the  unfortunate  patient 
through  the  entire  course  of  the  disease.  They  may  become  less  frequent  at 
the  height  of  the  fully  developed  tabes,  but  they  scarcely  ever  disappear 
entirely  and  frequently,  as  "tabes  dolorosa"  form,  at  least  subjectively,  the 
most  prominent  symptom.    But  in  additon  to  the  lancinating  pains,  the  radius 


252  DISEASES  OF  THE  SPINAL  CORD 

of  action  of  which  naturally  increases  with  the  progress  of  the  disease,  and 
which  to-day  attack  one  part  of  the  body  of  the  patient,  tomorrow,  another, 
we  meet  also  with  an  entirely  different  kind  of  pains — the  crises. 

The  term  crisis  includes  painful  sensations  of  acertain  "degree,"  mostly 
also  of  a  somewhat  longer  duration,  which  almost  invariably  proceed  from 
organs,  or  are  at  least  localized  in  them,  the  innervation  of  which  is  derived 
also  from  the  sympathetic  in  addition  to  the  cerebral  and  spinal  nerves 
(especially  of  the  vagus  group).  This  naturally  does  not  mean  that  these 
pains  are  transmitted  through  the  sympathetic — we  shall  speak  below  at 
greater  length  upon  this  point.  But  it  is  nevertheless  striking,  that  the 
organs  most  frequently  affected  (stomach,  bowels,  kidneys,  etc.)  according  to 
the  prevailing  views  (Lenander,  L.  R.  Mutter,  and  others)  and  investigations, 
are  susceptible  in  but  a  slight  degree  to  sensory  stimulations.  The  first  place 
is  beyond  a  doubt  to  be  assigned  to  the  stomach,  or  gastric  crises. 

The  typical  gastric  crisis  begins  almost  without  any  warning,  rarely  with 
slight  disturbances  of  the  stomach,  loss  of  appetite,  etc.  This  is  followed  by 
unusually  violent  pains  in  the  epigastrium,  which  resemble  the  pains  of 
gastric  ulcer,  but  may  become  more  colic-like;  they  are  mostly  described  as 
tearing,  jumping,  boring.  Almost  simultaneously,  the  patient  suffers  with 
uncontrollable  vomiting,  first  of  food,  then  of  mucus,  which  is  mostly 
stained  with  bile,  and  usually  shows  but  slight  acidity.  The  patients  vomit 
then  throughout  the  duration  of  the  crisis — usually  for  a  few  hours,  but  also 
with  interruptions  for  days  and  weeks — literally  all  the  nourishment  they 
take,  so  that  occasionally  artificial  feeding  must  be  resorted  to.  In  the  crises 
pain  and  vomiting  coincide,  but  the  attack  of  pain  may  persist  in  the  intervals 
between  vomiting.  The  loss  of  strength  of  the  patient  is  enormous  during 
this  time,  but  as  recovery  from  the  crises,  which  always  end  suddenly,  is  just 
as  quick,  the  conditions  are  rarely  so  bad  as  to  menace  life.  The  stomach 
crises  are  the  predominate  features  in  many  cases  of  tabes  and  recur  every 
few  weeks  or  months,  but,  as  a  rule,  only  during  a  period  of  the  disease,  which 
may  however  last  through  several  years.  The  majority  of  tabetics  are  fortu- 
nately spared  them,  but  the  percentage  of  those  affected  is  not  inconsiderable. 

The  other  forms  of  crises  occur  far  less  frequently. 

In  "intestinal  crises"  there  appear  at  intervals  profuse  attacks  of  diar- 
rhoea, usually  with  colic-like  pains. 

Attacks  simulating  renal  colic  have  been  described  as  kidney-crises. 
Crises  of  the  bladder,  urethra,  testicles,  clitoris,  are  all  analogous  conditions, 
which  the  patients  localize  in  the  organs  mentioned.  As  a  rule  they  are  of 
much  shorter  duration  than  the  gastric  crises;  those  affecting  the  genital 
region  are  frequently  accompanied  with  intense  feelings  of  libido,  as  well  as 
with  pain. 

Somewhat  more  frequent  are  the  laryngeal  crises.  The  onset  is  character- 
zed  by  an  apoplectiform  coughing  fit,  with  spasm  of  the  glottis,  which  may 


SYMPTOMATOLOGY  OF  TABES  253 

lead  to  serious  attacks  of  choking,  and  often  to  temporary  loss  of  conscious- 
ness. This  form  is  of  all  those  mentioned,  the  only  one  that  is  directly 
dangerous  to  life.  Attacks  of  tachycardia  and  pseudo-angina  pectoris  have 
been  described  as  pure  vagus  crises,  but  the  interpretation  is  absolutely 
uncertain. 

Between  the  crises  and  the  lancinating  pains  stand  the  neuralgias,  which 
are  comparatively  infrequent  but  obstinate.  These  neuralgias  may  attach 
themselves  to  the  cerebral  and  peripheral  nerves,  in  which  case  they  resemble 
rheumatic  neuralgias,  or  they  may  affect  the  internal  organs.  I  observed 
for  years  a  tabetic,  who  was  tortured  by  the  most  violent  unceasing  enteralgia, 
which  neither  increased  to  the  form  of  crises,  nor  ever  assumed  the  type  of 
lancinating  pains. 

To  the  subjective  sensory  disturbances  of  the  later  stages  correspond  more 
nearly,  than  in  the  early  stage,  objective  changes  of  sensation,  which  we  shall 
therefore  speak  of  now.  Even  before  the  hyperesthesia  has  completely 
risen  in  the  legs,  there  generally  appear  zones  of  sensory  disturbances  in  the 
trunk,  which  (Hitzig,  Laehr,  Dejerine)  have  segmentary  arrangement  (cf. 
general  diagnostics) ,  i.  e.,  occupy  the  region  of  the  skin,  supplied  by  the  indi- 
vidual posterior  roots,  resp.  the  segments  belonging  to  them.  Sometimes 
the  existence  of  such  zones  is  suggested  by  the  girdle  feeling  of  the  patient 
(even  in  the  region  of  the  girdle  pain  the  skin  may  be  hyperaesthetic!),  but 
often  the  girdle  zones  are  absolutely  independent,  at  the  height  of  the  breast 
or  the  costal  arch,  the  umbilicus,  etc.;  very  often,  they  are  broader  on  one- 
half  of  the  trunk  than  on  the  other,  or  are  demonstrable  only  on  one  side,  etc. 
The  form  of  disturbance  is  here,  usually  a  tactile  hyperaesthesia  or  anaesthesia 
with  a  most  evident  hyperalgesia,  also  with  diminution  of  the  cold  and  heat 
sensations.  Very  remarkable  also  is  the  disturbance  of  the  temperature  sense 
in  the  trunk  in  the  form  of  hyperesthesia  to  cold;  it  appears  usually  early  in 
the  disease,  often  in  the  first  stage,  and  the  patient  is  also  subjectively  con- 
scious of  it.  The  tabetic  becomes  unduly  susceptible  to  cold  touches, 
especially  to  cold  water,  but  also  to  other  cold  objects,  etc.  (usually  less 
frequently  to  cool  air!);  such  a  touch  almost  causes  him  pain,  he  starts  up, 
screams,  etc.  This  hyperaesthesia  to  cold  does  not  coincide  with  the  girdle 
zones,  which  have  just  been  mentioned,  but  generally  affects  the  trunk  from 
the  costal  arch  downwards,  often  also  the  upper  part  of  the  leg,  especially 
on  its  inner  side.  In  these  hyperaesthesias  to  cold  a  radicular  (segmentary 
type)  cannot  be  recognized.  This  disturbance  is  unusually  characteristic 
for  tabes,  and  is  rarely  absent  even  in  the  rudimentary  forms. 

Otherwise  hyperaesthesias  are  scarcely  ever  met  with  in  tabes.  On  the 
contrary  in  the  later  stage  the  loss  of  sensation  to  touch  ascends  the  legs, 
passes  over  trunk  and  arms  and  may  even  attack  face  and  head.  The  entire 
surface  of  the  skin  then,  or  only  the  surface  of  the  body  up  to  the  neck,  the 
breasts,  etc.,  shows  diminution  of  perception  to  a  simple  touch  and  usually 


254  DISEASES  OF  THE  SPINAL  CORD 

also  to  cold  (with  the  one  exception  named),  and  heat.  Total  anaesthesia, 
even  to  coarser  touch,  appears  only  late,  and  confines  itself  mostly  to  isolated 
parts  of  the  legs.  On  the  other  hand,  sensibility  to  pain  may  be  completely 
lost  even  at  an  early  stage.  Hypalgesia  in  feet  and  legs  is  found  almost 
invariably.  In  addition  to  this,  there  appears  very  soon  a  characteristic 
retardation  of  the  perception  of  pain  stimuli.  If  the  skin  be  pinched  or 
pricked,  the  patient  feels  at  first  merely  a  touch,  and  only  after  several  seconds 
does  the  feeling  of  pain  follow,  or  the  corresponding  pain  reflex,  the  with- 
drawal of  the  foot,  etc.  To  this  is  often  added  a  disturbance,  which  peculiarly 
supplements  hypalgesia,  the  so-called  summation  of  the  pain  stimulus.  A 
stimulus  of  only  short  duration  even  though  it  be  powerful  calls  forth  no 
feeling  of  pain,  but  a  longer  pinching,  or  pressing  the  point  of  the  needle 
against  the  skin,  for  a  definite  length  of  time  does.  In  addition  the  feeling  of 
pain  often  lasts  for  a  considerable  time  afterwards.  "  Abadie's  symptom," 
insensibility  to  pain  when  the  Achilles  tendon  is  pressed  upon  violently,  is  a 
very  inconstant  sign. 

Concerning  sensibility  of  the  internal  organs  in  tabes,  we  know  but 
little.  The  normally  great  sensitiveness  of  the  testicles  and  breasts  to 
pressure  disappears  (testicular  analgesia).  The  subjective  feeling  of  the 
need  of  emptying  the  bowels  and  bladder,  defecation  and  micturition,  is 
disturbed  early  (cf .  above) . 

Deep  sensibility  proper  is  always  more  or  less  influenced.  The  best 
known  disturbance  of  this  sort  is  the  Romberg  phenomenon,  generally  de- 
scribed as  a  disturbance  of  the  muscle  sense,  which  may  be  demonstrated  in 
90%  of  all  tabetics;  when  standing  with  feet  close  together,  and  eyes  shut, 
there  is  so  pronounced  a  swaying  of  the  entire  body,  that  the  patient  may 
even  fall  to  the  ground,  a  phenomenon  that  is  scarcely,  or  not  at  all,  per- 
ceived, when  the  eyes  are  open,  that  is,  when  the  patient  can  control  his  posi- 
tion with  his  own  eyes.  This  symptom  often  belongs  to  the  initial  stage  of 
tabes.  But  the  disturbance  is  by  no  means  sufficiently  well  explained  by  the 
otherwise  unfortunate  term  "disturbance  of  the  muscle  sense"  (Muskel- 
sinnstcerung) ,  for  in  it  participates  also  the  sense  for  passive  movements,  for 
gravity,  for  resistance,  as  well  as  finally  for  the  actual  perception  of  position. 

But  these  sensory  functions  may  be  tested  in  other  ways,  too,  and  show, 
as  has  been  said  before,  in  tabes,  as  a  rule,  great  changes  in  the  advanced' 
stages  of  the  disease.  The  patients  no  longer  feel  how  their  limbs,  toes,  etc., 
are  placed,  bent,  etc.,  by  the  examining  physician — disturbance  of  the 
perception  of  passive  movement;  without  the  control  of  their  eyes,  they  can 
not  move  their  feet  a  definite  distance  apart — disturbance  in  perception  of 
active  movement;  they  do  not  know,  where  their  legs  are  under  the  bed- 
cover— loss  of  perception  of  position.  In  similar  manner  the  senses  for 
weight  and  resistance  can  be  tested.  Naturally,  most  of  these  tests  prove 
the  presence  of  mixed  disturbances  of  the  different  sensory  faculties.    Pall- 


SYMPTOMATOLOGY  OF  TABES  255 

(Bsihesia  or  the  perception  of  vibration,  which  is  connected  with  the  sensitive- 
ness of  bones  (Egger),  is  also,  as  a  rule,  much  disturbed  in  tabes;  according 
to  some  authors  this  proceeds  parallel  with  the  ataxia  (see  below),  but 
according  to  Egger's  latest  report  this  does  not  seem  to  be  the  case.  The 
stereognostic  perception,  finally,  is  disturbed  more  rarely,  but  occasionally 
in  late  stages  it  is  very  marked;  the  patients  no  longer  recognize  an  object 
held  in  the  hand,  are  no  longer  able  to  take  money  from  their  purses,  etc. 

The  rather  frequent  absence  in  tabes  of  the  normal  feeling  of  fatigue 
after  movements  and  exertions,  is  probably  of  other  origin  (Frenkel). 

Partly,  at  least,  as  a  disturbance  of  the  sensory  pathways,  we  may  con- 
ceive the  symptom  we  have  not  yet  discussed  and  which  is  responsible  for 
the  name  given  tabes  during  its  entire  "second  stage":  ataxia.  Even 
if  there  are  cases  of  tabes  absolutely  without  ataxia,  this  complete  freedom 
from  it  is  very  rare;  its  presence  in  tabes  is  extraordinarily  significant  and 
characteristic,  as  nowhere  else  in  human  pathology  do  we  meet  it  in  exactly 
the  same  form  and  but  rarely  in  a  similar  form.  If  the  tabetic — in  any 
position  desired— is  asked  to  perform  complicated  movements,  especially 
with  the  extremities,  there  appears  an  uncertainty,  a  wavering;  the  move- 
ments wander  from  their  direct  line  and  proceed  in  zig-zag  fashion.  If  the 
patient  is  to  bring  the  tips  of  his  index  fingers  together,  by  bringing  the  arms, 
which  have  been  stretched  far  to  the  side,  towards  the  front,  this  movement 
is  attended  by  utterly  unregulated  waverings,  extending  on  all  sides  about 
the  axis  of  the  movement.  He  invariably  spills  water  from  a  full  glass 
that  he  is  to  take  to  his  lips;  in  writing,  one  letter  becomes  microscopic- 
ally small  while  to  form  the  next,  the  hand  moves  the  pen  all  the  way  across 
the  page.  He  is  not  able,  when  lying  on  his  back,  to  perform  the  more 
delicate  movements  with  his  feet,  to  describe  a  circle  with  the  points  of  his 
foot,  to  write  numbers  in  the  air,  to  place  the  heel  of  one  foot,  moving  it 
through  the  air,  upon  the  knee  of  the  other  leg.  If  he  is  asked  to  walk  upon 
a  line,  he  finds  it  very  difficult,  because  his  feet  continually  stray  to  one  side 
or  the  other.  On  the  whole  the  gait  of  the  ataxic  is  peculiarly  characteristic. 
At  first  it  is  swinging  and  stamping  probably  with  the  knee  raised  unneces- 
sarily high  and  toes  pointing  downwards  (like  a  rooster's  walk).  Later  the 
legs  spread  apart,  usually  assisted  by  a  cane,  which  is  placed  far  in  front,  in 
order  thus  to  attain  as  broad  a  base  as  possible  for  the  three  points  of  support. 
Even  a  slight  degree  of  so-called  static  ataxia  is  occasionally  present,  in 
which  the  patient  finds  it  less  possible  to  sit  or  stand  perfectly  erect,  without 
at  the  same  time  moving  the  muscles  of  the  trunk,  but  far  more  frequent 
is  the  ataxia,  which  has  just  been  discussed,  the  locomotor  (or  movement) 
ataxia,  which  in  England  and  France  has  given  the  disease  its  name.  All 
these  atactic  movements,  moreover,  occur  even  more  typically  when  the 
eyes  are  closed,  so  that,  to  a  certain  extent,  even  the  Romberg  symptom, 
just  mentioned,  may  be  considered  a  partial  phenomenon  of  ataxia. 


256  DISEASES  OF  THE  SPINAL  CORD 

Ataxia  is  to  be  understood  as  a  disturbance  of  co-ordination,  the  main 
cause  of  which  must  be  sought  in  a  confusion  of  the  centripetal  stimuli — in 
the  widest  sense,  therefore,  of  the  sensory  stimuli,  though  the  disturbance, 
at  first  sight,  appears  to  be  a  motor  one. 

Genuine,  purely  motor  symptoms,  however,  are  very  infrequent  even 
in  advanced  stages  of  tabes.  In  the  greater  number  of  grave  cases  of  tabes, 
it  is  true,  the  patients  are  forced  to  stay  in  bed,  and  there  is  to  a  high  degree, 
a  feeling  of  general  weakness,  but  this  is  altogether  the  result  of  sensory 
disturbances  and  ataxia.  To  some  extent  also  trophic  disturbances  prevent 
motion.  Paralyses  are  known  almost  only  in  the  area  supplied  by  the 
cerebral  nerves,  if  we  leave  out  of  consideration  the  rare  combinations  with 
atrophic  paralyses,  described  by  Dejerine  and  others,  which  are  probably 
partly  dependent  upon  implication  of  the  peripheral  motor  nerves,  partly 
the  result  of  disease  of  the  gray  anterior  horns,  and  which,  therefore,  in 
neither  case,  belong  to  the  picture  of  tabes  proper.  In  these  cases,  we  find 
conditions  of  weakness  and  atrophy,  especially  in  the  small  muscles  of  the 
feet  and  hands,  in  the  area  of  the  peroneal,  as  well  as  in  those  of  the  ulnar 
and  median  nerves;  secondary  contractures,  the  pied  bot  tabetique  (tabetic 
club  foot),  claw  and  monkey  hand  are  the  consequences.  The  reaction  of 
degeneration  is  not  demonstrable  in  the  majority  of  the  cases.  But  the 
general  emaciation,  so  frequently  observed  in  the  terminal  stage  of  tabes,  is 
only  rarely  due  to  these  changes,  much  more  frequently  it  depends  upon 
disturbances  in  nutrition,  and  disuse  of  the  muscles  (marantic  tabes). 
More  frequent  are,  as  has  been  mentioned,  the  paralyses  of  individual 
cerebral  nerves. 

The  olfactory  is  affected  only  in  very  rare  instances. 

The  disturbances  of  the  optic  nerve — amblyopia,  diminution  of  the 
visual  field,  white  atrophy- — have  been  mentioned;  they  are  not  at  all 
common. 

The  oculomotor,  trochlear  and  abducent  nerves  are  more  frequently  and 
also  to  a  more  considerable  degree  affected  than  the  other  nerves;  this  also 
has  been  mentioned  above.  Outside  of  the  paralyses  of  individual  nerves 
and  the  ophthalmoplegias,  there  occur  also  accommodation  paralyses — 
scarcely  ever  isolated — as  well  as,  but  rarely,  irritation  phenomena  (con- 
vergence spasm)  which  like  the  club  foot  in  tabes  may  also  be  considered 
as  paralytic  contractures — possibly  with  better  reason.  The  appearance, 
however,  of  " disturbing"  double  images,  is  by  no  means  very  frequent,  in 
even  the  serious  forms  of  tabetic  paralyses  of  the  ocular  muscles,  since  the 
patients  are  often  able  to  suppress  them. 

Disease  of  the  trifacial  causes  frequently,  in  advanced  cases,  more  or  less 
severe  disturbances  of  the  cutaneous  sensibility  in  the  face,  particularly  in 
the  conjunctival  mucous  membrane,  which  especially  when  associated  with 
lagophthalmos  (this  is,  of  course,  rare)  may  lead  to  the  formation  of  ulcers, 


THE  SYMPTOMATOLOGY  OF  TABES  257 

and  ophthalmia.  The  radicular  (segmental)  type  may  be  recognized  here 
also.  Very  rarely  the  motor  trifacial  is  affected  causing  weakness  in  the 
muscles  of  mastication. 

In  the  facial,  paralyses  as  well  as  spasms  (tic  convulsif)  have  curiously 
enough  been  described  very  infrequently,  but  both  occur. 

The  auditory,  again,  is  more  frequently  affected  and  labyrinthine  vertigo 
as  well  as  disturbances  in  hearing  are  described,  alone  and  together,  that  is, 
disturbances  that  include  part,  or  the  whole  of  the  Meniere  symptom  com- 
plex. In  the  largest  statistical  paper  (Tumpowsky  in  225  cases  of  tabes),  a 
proportion  of  0.9%  of  affections  of  the  auditory  nerve  was  given,  a  figure 
which  probably  is  too  small,  and  could  certainly  be  raised  by  exact  otological 
examination. 

Glosso-pharyngeal  Vagus. — Of  taste  disturbances  all  varieties  are  reported, 
though,  on  the  whole,  they  are  of  rare  occurrence.  Absolute  ageusia,  loss 
of  the  sense  of  taste,  as  well  as  confusion  of  the  separate  qualities  of  taste, 
occurs.  Severely  affected  tabetics  frequently  say  that  everything  tastes  to 
them  "like  wood";  with  closer  investigation,  however,  there  is  proved  to  be 
only  a  partial  lowering  of  the  separate  qualities  of  taste.  One  observation 
of  Pfeifer  mentions,  on  the  analogy  of  the  delayed  pain  sensation,  a  retarded 
taste  sensation.  This  group  of  phenomena,  as  a  whole,  belongs  to  the 
domain  of  atypical  forms  of  tabes,  especially  to  those  of  the  bulbar-paralytic 
type. 

A  pure  vagus  symptom  is  the  not  infrequent  tachycardia  of  tabetics;  at 
rest,  the  pulse  has  been  observed  as  high  as  120.  Tachypncea  may  belong 
here  also.  The  disturbances  of  the  laryngeus  inferior — seldom  permanent 
paralyses,  most  frequently  posticus  paralysis,  occasionally  spasms,  now  and 
then  the  paroxysmal  pareses  of  the  laryngeal  crises — lead  us  to  the  crises 
that  belong  almost  essentially  to  the  vagus  group,  and  which  were  discussed 
above.  Here  we  must  emphasize  the  fact,  that,  if  we  wish  to  consider  the 
gastric  crises,  etc.,  as  vagus  symptoms,  we  must  assume  in  numerous  cases 
an  early  vagus  affection  almost  parallel  to  that  of  the  opticus.  I  believe, 
therefore,  as  has  been  mentioned  already,  that  part  of  the  crises  must  cer- 
tainly be  due  to  affections  of  the  sympathetic  nerve. 

Spinal  Accessory  Symptoms. — Pareses  of  the  cucullaris  and  the 
sterno-cleido-mastoid  have  recently  been  described  by  Seiffer;  they  must 
belong,  at  any  rate,  to  the  rarities,  as  do  also  the  affections  of  the  hypo- 
glossus,  which  occur  occasionally  accompanied  by  atrophy. 

One  unusually  frequent  motor  symptom,  the  loss  of  muscle  tone,  we  have 
not  as  yet  spoken  of.  The  reflex  inhibitions,  normally  present  in  every 
active  muscular  innervation,  which  prevent  the  movement  from  going  beyond 
its  goal  are  often  lost  early  in  tabes  (nearly  always  in  the  final  stages).  There- 
fore the  purposive  movements,  when  the  strength  is  normal  (even  when 
ataxia  is  not  present),  make  an  excessive  impression,  which  is  specially 
17 


258  DISEASES  OF  THE  SPINAL  CORD 

increased  by  every  passive  movement.  Without  any  trouble  and  without 
any  sensation  of  pain  the  legs  of  the  patient  may  be  flexed  to  such  a  degree 
as  to  touch  the  head;  the  hyperextensibility  of  the  fingers  and  wrist  often 
makes  it  possible  to  bring  the  fingers  passively  into  contact  with  the  dorsal 
surface  of  the  forearm;  the  leg  extended  at  the  knee,  with  the  patient 
standing  upright,  is  bent  backward  by  the  weight  of  the  body  (genu  recurva- 
tum).  At  the  same  time  the  muscles,  ligaments,  joints  are  anatomically, 
as  a  rule,  perfectly  normal.  (Of  course  trophic  changes  in  the  joints  may 
greatly  increase  the  extent  of  passive  movement.) 

Electrical  changes  in  the  muscles  are  naturally  to  be  expected  only  when 
peripheral  paralyses  exist  and  have  been  so  reported. 

Of  the  motor  irritation  phenomena,  athetosis  is  a  not  very  infrequent  symp- 
tom of  irritation  in  advanced  tabes,  in  tabes  superior,  and  in  the  bulbar- 
paralytic  form;  it  gives  the  complete,  uni-  or  bilateral,  clearly  defined  picture 
of  the  movement  disturbances  just  as  they  are  observed  in  other  cerebral  dis- 
eases :  continuous,  slow,  worm-like  movements,  which  may  lead  to  apparent 
dislocations,  to  overextensions  and  to  quite  grotesque  positions,  especially  in 
the  fingers  and  arms.  Contractures  and  their  concomitant  symptoms — ex- 
aggeration of  the  reflexes,  Babinski  symptom,  etc. — are  observed  only  in  the 
combination  of  tabes  with  other  spinal  troubles.  That  the  crises,  especially 
the  laryngeal,  represent  partly  paralyses,  partly  also  spasmodic  spastic  con- 
ditions, has  been  said  above. 

Genuine,  pure  sympathetic  symptoms — widening  of  the  palpebral  fissure, 
unilateral  blushing  and  sweating,  etc. — have  been  found  only  exceptionally 
in  tabes. 

The  change  of  the  tendon  reflexes,  which  characterizes  even  the  initial 
stage  of  tabes,  is  practically  constant  in  the  ataxic  stage.     The  Achilles  and 
patellar  tendon  reflexes  are  those  most  frequently  absent;  the  patellar  reflex 
can  only  very  rarely  be  evoked  even  by  artificial  reenforcement  (Jendrassik). 
The  triceps  and  the  lower  jaw  reflex  are  constantly  absent  in  tabes  superior 
but  are  not  necessarily  absent  even  in  advanced  cases  of  the  usual  type, 
beginning  with  the  legs,  since  the  reflex  arcs  for  the  arms  and  the  head  may 
remain  undisturbed  for  a  long  time.     Occasionally,  however,  the  tendon 
reflexes  that  have  been  lost,  may  return  temporarily  or  permanently,  without 
a  cure  being  effected,  as  in  slight  narcosis,  after  cerebral  apoplexies,  and 
upon  the  advent  of  other  systemic  diseases.     Occasionally  the  reappearance 
seems,  symptomatically,  to  show  a  certain  improvement,  especially  when 
there  was  originally  only  a  difference  in  the  reflexes  on  the  two  sides,  and  then, 
one  disappeared  to  return  again.     This,  at  any  rate,  does  not  seem  to  be 
such  a  very  rare  occurrence.     In  patients,  moreover,  in  whom  the  tabes, 
from  the  beginning,  appeared  in  the  form  of  a  combined  systemic  disease,  a 
tabo-paralysis,  etc.,  the  reflexes  can  be  retained  from  the  first,  or  even 
increased. 


THE  SYMPTOMATOLOGY  OF  TABES 


259 


The  cutaneous  reflexes  are  altered  very  much  more  rarely,  most  frequently 
the  plantar.  This  is  lost  in  most  tabetics,  as  soon  as  the  sensory  disturbances 
in  the  feet  have  attained  a  certain  degree;  their  decrease  is  parallel,  on  the 
whole,  with  the  loss  of  feeling  (which  by  no  means  holds  good  for  the  tendon 
reflexes) .  The  so-called  Babinski  dorsal  toe  reflex  is  never  present  in  pure 
tabes.  The  cremaster  reflexes,  and  especially  the  abdominal  reflexes  are,  on 
the  other  hand,  only  exceptionally  affected  by  the  tabetic  process,  even 
where  zones  of  anaesthesia  exist  in  the  trunk.  Whether  the  activity  of  the 
abdominal  reflexes,  which  is  not  rare,  is  connected  with  the  hyperesthesia  to 
cold  in  that  region,  has  not  yet  been  ascertained. 

Barring  the  pupillary  reflex  already  mentioned,  the  so-called  "internal 
reflexes"  (of  swallowing,  sneezing,  etc.),  are  disturbed  only  exceptionally, 
unless  we  are  willing  to  accept  some  of  the  crises,  as  the  laryngeal  crises,  etc., 
as  due  to  reflex  overexcitability,  a  theory  which  certainly  would  not  hold  good 
for  the  majority  of  cases.  Formerly  several  forms  of  male  impotence  were 
explained  as  due  to  reflex  disturbance  (faulty  ejaculation). 

The  symptoms  referable  to  the  urogenital  tract  in  tabes,  on  the  whole, 
present  a  mixture  of  motor,  sensory,  and  reflex  disturbances;  they  have 
already  been  discussed  in  part  with  the  early  symptoms. 

In  the  completely  developed  clinical  picture  of  the  disease,  they  are 
rarely  ever  altogether  absent,  and  the  bladder  disturbances  especially,  very 
frequently  are  of  great  importance  in  the  termination  of  the  disease,  causing 
death  by  ascending  pyelonephritis. 

The  bladder  disturbances  are  also  the  most  frequent  disturbances  in  this 
group.  The  patients  must  in  many  cases  use  a  greater  amount  of  strength 
to  empty  the  bladder,  and  there  usually  remains  some  residual  urine — retentio 
urinae;  this  is  mostly  caused  by  spasm  of  the  sphincter,  but  occasionally 
there  is  also  simultaneous  weakness  of  the  detrusor.  This,  however,  is  not 
constant;  it  may  even  happen  in  tabes,  that,  because  of  lasting  spasm  of  the 
sphincter,  a  genuine  hypertrophy  of  the  bladder,  a  so-called  "board-bladder" 
(Balkenblase)  may  develop  with  partial  hypertrophy  of  the  muscle  of  the 
detrusor.  The  retention  may  lead  to  the  clinical  picture  of  ischuria-para- 
doxa :  the  constantly  filled  bladder,,  unable  to  empty  itself,  emits  passively,  so 
to  say,  small  quantities  continuously,  like  an  overflowing  rain-barrel.  Nat- 
urally such  patients  very  often  take  shelter  in  the  last  resort — the  catheter; 
usually  the  bladder  becomes  infected  from  this,  sooner  or  later,  so  that 
cystitis  in  grave  cases  of  tabes  is  often  found  as  a  direct  result  of  this. 
This,  however,  is  also  caused  by  the  opposite  condition  to  retention;  namely, 
incontinence  of  urine — the  relaxation  of  the  sphincter  with  constant  drip- 
ping of  urine.  In  this  condition  also,  which  is  almost  as  frequent  as  reten- 
tion, infectious  micro-organisms  easily  find  their  way  through  the  urethra 
into  the  insufficiently  closed  off  bladder.  Disturbances  of  the  intestinal 
sphincters  are  found  less  frequently ;.   futhermore  all  proof  for  the  statement 


26o  DISEASES  OF  THE  SPINAL  CORD 

that  the  constipation,  which  is  as  frequent  in  tabes  as  in  other  diseases  of 
the  central  nervous  system,  is  induced  by  a  spasm  of  the  sphincter  is  lack- 
ing. It  is  easier  to  believe  that  it  is  occasionally  caused  by  a  chronic 
paresis  of  the  intestinal  muscles,  or  a  genuine  ataxia,  which,  however, 
frequently  seems  to  affect  the  sphincters  alone.  Often  patients  are 
troubled  by  the  lack  of  control  over  the  emission  of  urine  or  of  evacuation 
of  feces,  due  to  a  deeply  seated  anaesthesia. 

Impotence  is  one  of  the  most  frequent  tabetic  symptoms  of  this  group, 
known,  as  such,  even  to  the  layman.  Almost  all  male  patients — in  women 
disturbances  of  libido  and  orgasm  are  seldom  even  observed  by  the 
patients  themselves — show,  in  advanced  stages  of  tabes,  a  decline  in  virile 
power,  which  affects  all  the  factors  of  this  function;  erection  and  ejaculation 
in  particular  become  insufficient,  take  place  prematurely,  or  are  even  pain- 
ful, whereas  the  libido,  to  the  torture  of  the  patient,  may  last  for  some  time 
after  the  loss  of  potency.  In  the  final  stage  there  is  generally  total  im- 
potence. In  the  beginning  of  the  disease,  on  the  other  hand,  the  presence 
of  satyriasis  may  be  noted. 

Of  the  ordinary  groups  of  tabetic  symptoms,  only  the  trophic  dis- 
turbances and  the  changes  in  the  cerebro-spinal  fluid  remain  to  be 
mentioned. 

The  trophic  disturbances  may  affect  all  the  tissues  of  the  body,  but  most 
characteristically,  the  epidermis  and  the  skeletal  system.  The  deficient 
nourishment  of  the  skin  is  betokened  frequently  by  ulcers  and  bed-sores; 
wherever  a  tight  bandage,  a  set  of  false  teeth,  a  urine  bottle  have  pressed 
for  some  time,  ulcers  appear,  which  occasionally  become  very  deep,  have  but 
a  slight  tendency  to  heal  and  are  nearly  always  painless.  Such  an  ulcer, 
which  appears  in  tabetics  apparently  spontaneously,  is  the  umal  perforant" 
usually  coming  on  gradually  on  the  under  surface  of  the  great  toe,  or  of  the 
ball  of  the  foot  back  of  the  great  toe,  which,  as  a  rule,  eats  in  very  deeply, 
even  to  the  bone,  and  is  difficult  to  heal.  Also  an  (apparently)  spontaneous 
herpes  zoster  is  often  observed  in  tabetics.  Still  more  striking  are  the 
trophic  disturbances  in  the  bones  and  joints,  more  particularly  the  arthro- 
pathies (Fig.  60).  Suddenly,  without  any  spontaneous  pains,  and  within  a 
few  days,  any  joint  of  the  body  may  become  swollen;  the  patient,  as  a  rule, 
does  not  notice  it  until  it  causes  purely  mechanical  disturbances  in  motion. 
Upon  objective  examination,  however,  one  generally  finds  serious  changes; 
of^en  an  enormous  distention  at  the  ends  of  the  bones  and  effusion  into  the 
joint.  Local  heat  and  redness,  as  well  as  pain,  are  absent.  An  X-ray 
examination  shows  an  indistinct  clearing  up  of  the  shadows  of  the  bones, 
which,  however,  retain  their  sharp  contours;  the  anatomic  examination  often 
shows  detachment  of  the  periosteum  with  softening  of  the  parts  of  the  bone 
beneath,  formation  of  osteophytes,  disintegration  of  small  particles  of  bone, 
destruction  of  ligaments  and  joint  cartilage.     The  exudate  is,  as  a  rule, 


SYMPTOMATOLOGY  OF  TABES  261 

serous  and  but  rarely  contains  pus.  The  consequences  are  loose  joints, 
subluxations,  etc. ;  because  of  the  chronic  course  and  the  very  slight  tendency 
to  heal,  permanent  deformities  often  supervene,  which,  combined  with  other 
tabetic  symptoms,  especially  with  hypotonia  of  muscles,  may  lead  to  almost 
grotesque  pictures  of  diseases,  as,  for  instance,  to  the  not  infrequent  genu 
recurvatum  with  synchronous  arthropathies.  As  a  matter  of  fact,  the 
arthropathies  are  found  most  frequently  in  the  knee-joints,  then  come  the 


Fig.  60. — Arthropathy  of  the  right  knee  in  tabes  dorsalis. 

hip-,  the  shoulder-,  the  ankle-joints,  etc.  In  more  recent  times,  their  occur- 
rence in  the  spinal  column  has  been  described  with  striking  frequency; 
probably  the  general  employment  of  radioscopy  may  have  contributed  to  this. 
To  a  certain  extent  the  abnormal  fragility  of  the  bones  in  tabes,  runs  parallel 
with  the  arthropathies;  even  slight  exertion  leads  to  complete,  though  pain- 
less transverse  fractures  of  the  long  bones,  which,  however,  show  a  greater 
tendency  to  heal  than  the  arthropathies.  Dentists,  moreover,  frequently 
observe  a  strikingly  rapid  deformation  of  the  jaws,  especially  of  the  alveolar 
processes,  with  falling  out  of  the  teeth;  the  process  resembles  absolutely  the 


262  DISEASES  OF  THE  SPINAL  CORD 

atrophy  of  the  senile  jaw,  but  is  more  rapid  and  appears  earlier.  The 
change  often  takes  place  so  quickly  that  sets  of  false  teeth,  made  to  fit  the 
mouth,  no  longer  fit  properly  at  the  end  of  a  few  months. 

The  last  symptom  which  must  be  included  among  those  that  are  almost 
constant  in  tabes,  is  the  change  in  the  cerebrospinal  fluid.  If  a  few  cubic 
centimeters  of  the  liquid  are  withdrawn,  in.  the  usual  way,  by  lumbar  punc- 
ture, from  the  spinal  canal  of  a  tabetic,  and  subjected  to  centrifugalization, 
there  is  regularly  found  in  the  sediment  an  increase  of  the  normally  rather 
rare  cellular  elements,  and  especially  of  the  lymphocytes,  so  called  because 
of  their  likeness  to  the  lymphocytes  of  the  blood;  there  is  usually  a  very  high 
degree  of  "lymphocytosis"  of  the  fluid  (Widal,  Sicard,  Schoenborn,  Nissl). 
A  similar  change  is  found  in  pure  meningitis,  as  well  as  in  some  other  meta- 
syphilitic  diseases  of  the  central  nervous  system- — progressive  paralysis, 
cerebro-spinal  syphilis.  The  most  likely  interpretation  of  this  symptom  in 
tabes  is  a  chronic  change  in  the  meninges  brought  about  by  the  former 
attack  of  syphilis — a  syphilosis  of  the  meninges.  The  supposition,  that  the 
former  attack  of  syphilis  in  itself  causes  the  lymphocytosis  in  tabetics — in  a 
certain  number  of  cases  similar  findings  have  been  made  in  secondary 
syphilis — becomes  improbable,  since  upon  closer  investigation  of  the 
meninges  in  tabes,  anatomic  changes  are  now  almost  regularly  found 
{Nageotte,  cf.  above).  Lymphocytosis  is  an  unusually  constant  and  early 
symptom  of  tabes  dorsalis. 

There  are  several  groups  of  symptoms,  which  combine  now  and  then  with 
those  of  tabes  dorsalis.  Among  these  are  the  symptoms  caused  by  the 
participation  of  those  systems  of  tracts  or  columns  in  the  spinal  cord  which, 
as  a  rule,  are  not  affected  in  tabes,  especially  that  of  the  pyramidal  tracts. 
Then  we  have  before  us  the  complete  picture  of  "combined  system  disease," 
usually  accompanied  by  spasticity  and  exaggerated  reflexes.  The  combina- 
tion with  peripheral  neuritis,  also,  is  not  infrequent.  The  most  frequent 
concomitant  disease  of  the  nervous  system  is  probably  progressive  paralysis. 
But  this,  as  Erb  pertinently  emphasizes,  must  not  be  interpreted,  as  if  tabes, 
when  reaching  a  certain  high  level,  very  frequently  passed  over  into  progress- 
ive paralysis;  this,  on  the  contrary,  is  a  relatively  rare  occurrence.  The 
combination  is,  in  the  first  place,  rather  an  anatomical  one — in  autopsies  of 
paretics  a  gray  degeneration  of  the  posterior  columns  (Westphal)  is  often 
found,  and  since  then  it  has  been  learned  that  occasional  tabetic  symptoms 
(Romberg,  pupillary  rigidity,  slight  disturbances  of  sensation)  may  very 
often  be  found  in  paretics  upon  closer  investigation.  Whether,  in  such  a 
case,  the  tabes  was  superimposed  on  a  paresis  already  present,  or  whether 
the  two  developed  simultaneously,  we  are  generally  unable  to  decide;  either 
might  happen.  But  tabo-paresis,  though  a  frequent  disease  picture,  is  not 
nearly  as  common  as  simple  paresis,  and  especially  not  nearly  so  fre- 
quent as  simple  tabes.     Otherwise,  psychic  symptoms  in  tabes  are  rare,  but 


A  NA  TO  MIC  CHA  NGES  AND  S  YMP  TOMS  2  63 

combinations  with  so-called  "neuroses,"  epilepsy  or  Basedow's  disease 
may  occasionally  be  observed. 

We  have  already  stated  that  tertiary  syphilis  may  occur  with  tabes.  But 
in  particular  we  see  certain  late  forms  of  visceral  syphilis  in  tabes,  especially 
aneurism  of  the  aorta,  to  such  an  extent,  that  Babinski  has  described 
a  peculiar  symptom  complex:  aneurism  of  the  aorta,  with  disturbances  of 
the  tendon  and  pupillary  reflexes.  In  the  great  majority  of  cases,  the 
aneurism,  as  well  as  the  not  infrequent  insufficiency  of  the  aortic  valves, 
might  be  caused  by  a  syphilitic  aortitis. 

The  occurrence  of  chronic  gastro-intestinal  disturbances  and  ascending 
pyelonephritis  in  tabes,  is  explained  by  the  foundation  for  these  troubles  by 
the  tabetic  disturbances  of  the  respective  sphincters. 

How  far,  then,  can  a  pathogenesis  of  the  tabetic  symptoms  be  estab- 
lished, how  far  can  they  be  traced  back  to  the  anatomic  process?  This 
question  is  easily  answered  in  regard  to  some  phenomena;  for  others  up  to 
this  time  with  great  difficulty,  or  not  at  all.  The  objective,  and  to  an  extent, 
the  subjective  sensory  disturbances  are  founded,  doubtless,  upon  a  degenera- 
tion of  the  posterior  root  fibres;  this  is  proved  in  many  cases  by  the  complete 
radicular  distribution,  and  the  ascent  of  the  disturbances,  etc.  But  even 
here,  much  is  left  unexplained.  Thus,  for  instance,  the  "sensory  disturb- 
ances of  the  internal  organs,"  especially  of  the  intestines  and  bladder,  seem 
to  be  directly  or  indirectly  connected  with  degeneration  of  sympathetic 
tracts.  Also  the  reason  why  so  frequently  certain  qualities  of  sensation 
are  disturbed  more  seriously  or  earlier  than  others,  has  not  been  definitely 
established  thus  far.  The  explanation  of  the  tabetic  pains,  especially  of  the 
lancinating  pains,  and  of  the  so-called  crises,  is  most  difficult.  For  the 
former,  which  show  a  predilection  to  limit  themselves  to  the  area  of  a 
peripheral  nerve,  it  is  possible  to  consider  irritation  or  compression  of  a 
circumscribed  place  of  the  posterior  roots,  even  though  the  type  of  pains, 
which  certainly  depends  on  such  compression  (in  pachymeningitis,  tumors, 
herpes  zoster) ,  resemble  but  slightly,  as  a  rule,  the  lancinating  pains.  Finally 
as  to  the  crises,  we  know,  at  present,  no  anatomic  basis.  Their  localization, 
their  character,  their  connection  with  considerable  increases  in  blood  pres- 
sure, tempt  us  very  much  to  refer  them  to  the  vasomotor  nerves,  i.  e.,  to  the 
sympathetic  nerve  system. 

The  disturbance  of  the  muscle  sense,  Romberg's  phenomenon,  probably 
must  be  traced  back  also  to  a  disturbance  of  the  centripetal  paths,  most 
likely  of  the  fibres  ascending  in  the  posterior  column;  pallesthesia  and  its 
disturbance  belong  here  also,  and  for  the  stereognostic  disturbances  we 
must  assume,  in  contradistinction  to  the  astereognosia  of  cerebral  affections, 
a  mixture  of  centripetal  and  centrifugal,  sensory  and  motor  disturbances. 
On  the  other  hand,  the  frequent  absence  of  the  feeling  of  fatigue  in  tabetics, 


264  DISEASES  OF  THE  SPINAL  CORD 

represents  certainly  a  pure  disturbance  in  the  sensory  rapport  of  the  extrem- 
ities, that  is,  in  the  posterior  columns. 

The  understanding  of  tabetic  ataxia  has  long  caused  great  difficulties. 
If  we  consider  briefly  its  manifestations,  we  shall  see  immediately  that  a 
motor  disturbance  is  out  of  the  question.  The  patient  is  able  to  properly 
innervate  every  single  muscle,  at  least  with  the  aid  of  the  control  of  the  eye; 
the  impulse  of  the  will  to  the  periphery,  i.  e.,  the  motor  conduction  is  therefore 
absolutely  retained  in  the  usual  forms  of  tabetic  ataxia.  What  is  disturbed 
is, first, the  sensory  rapport  at  the  periphery,  the  "information"  of  the  central 
organ  as  to  the  position  of  the  separate  divisions  of  limbs,  and  skeletal  mus- 
cles, but  then  again,  the  synergism  of  the  individual  muscles,  especially  the 
relation  between  agonist  and  antagonist.  For  the  lack  of  sensory  rapport, 
we  have  the  anatomic  basis:  the  degeneration  of  the  posterior  columns  (in 
the  broadest  sense) ;  for  the  co-ordinations,  we  should  have  to  consider,  the 
so-called  co-ordination  centers  in  the  cerebellum,  pons,  quadrigeminal  bodies; 
they  are  certainly  not  affected,  for  the  most  part,  in  tabetic  ataxia.  But 
co-ordination,  also,  needs  the  centripetal,  sensible  (sensory)  impulses;  it  is 
natural,  therefore,  to  assume  here,  too,  a  genuine  disturbance  of  the 
centripetal  paths.  Without  entering  here  upon  the  moot  points  in  detail 
(which,  in  Erb's  opinion,  are  not,  as  yet,  fully  cleared  up),  let  us  mention 
only  that  hypotonia  of  the  muscles  (cf.  below)  as  well  as  reflex  processes 
seem  to  play  some  part  in  tabetic  ataxia.  But  in  the  main,  it  is  doubtless 
a  question  of  a  "sensory"  ataxia  (Ley den)  and  of  a  disturbance  of  the  sensory 
paths  (Otfried  Fbrster) .  The  last  named  author  sees  the  anatomic  basis  of 
tabetic  ataxia  in  the  degeneration  of  the  reflex  collaterals  in  the  spinal 
cord,  the  collaterals  to  Clarke's  columns  (cerebellum)  and  the  long  fibres  of 
the  posterior  column.     I  fully  agree  with  his  point  of  view. 

The  disturbance  of  the  tendon  reflexes  is  certainly  founded  upon  the 
degeneration  of  the  reflex  collaterals,  of  the  root  fibres  passing  from  the 
radicular  zone  into  the  gray  posterior  columns.  (Level  for  the  patellar  re- 
flexes: lumbar  segments  II  to  IV;  Achilles  tendon  reflexes:  sacral  segments 
III  to  V;  triceps  reflex:  cervical  segment  VI-VII.)  Hypotonia,  also,  per- 
haps even  the  disturbances  of  the  cutaneous  reflexes  (rare)  are  to  be  traced 
back  to  the  reflex  collaterals.  For  the  "internal  reflexes"  and  the  disturb-, 
ances  of  the  urogenital  tract  (the  so-called  sphincter  disturbances,  incon- 
tinence and  retention  of  feces  and  urine,  impotence),  the  genesis,  as  we 
mentioned  above,  is  not  absolutely  clear.  We  know,  to  be  sure,  that  the 
lumbar  segments  are  the  seat  of  the  corresponding  centers.  But  animal 
experiments  seem  to  indicate  that  the  path  for  the  processes  mentioned 
passes  at  least  in  part  through  the  sympathetic,  for  the  co-affection  of 
which  in  tabes  dorsalis  unfortunately  only  a  limited  number  of  reports  are 
available. 


CLINICAL  COURSE  AND  PROGNOSIS  IN  TABES  265 

The  motor  centers  and  paths  need  occupy  us  less.  In  the  rare  atrophic 
paralyses,  corresponding  lesions  of  the  anterior  horn  have  been  found,  and 
here  and  there  also  neuritic  changes.  Of  the  irritation  phenomena,  athetosis, 
according  to  the  present  state  of  our  knowledge,  is  certainly  a  cerebral 
symptom.  In  the  phenomena  of  the  cranial  nerves  (nerves  of  the  eye  muscles, 
special  sense  nerves,  trigeminus,  vagus)  there  appear  with  about  the  same 
frequency,  neuritic  and  nuclear  lesions;  optic  atrophy  especially  is,  as  stated 
above,  before  all  a  sign  of  a  peripheral  affection. 

The  changes  in  the  pupils,  especially  the  reflex  light  rigidity,  have  always 
given  much  concern  to  neurologists  and  ophthalmologists.  Experimental 
investigations  (Bach  and  H.  Meyer)  seemed  to  prove  that  in  the  posterior 
columns,  near  the  fourth  ventricle,  that  is  in  the  uppermost  cervical  segment, 
there  is  a  center  for  the  pupillary  reflex  (and  for  the  origin  of  so-called  "spinal 
miosis");  but  recently  Bumke  has  found  proof  to  the  contrary,  and  now  it 
almosts  seems,  as  if  one  should  have  to  return  to  the  older  theories,  according 
to  which,  pupillary  rigidity  is  to  be  viewed  as  a  lesion  in  the  pupillary  fibres 
of  the  optic  nerve,  in  the  nuclei  of  the  oculomotor  or  in  the  ciliary  ganglion. 
But  no  certain  results  have  been  established  for  the  localization  of  this 
apparently  simple  symptom  (i.  e.,  for  the  anatomic  proof). 

For  the  trophic  disturbances,  to  be  brief,  we  have,  at  present,  absolutely 
no  satisfactory  localization  in  the  central  nervous  system ;  only  this  is  certain, 
that  they  are  not  to  be  understood  only  as  local,  idiopathic  destructive  proc- 
esses of  the  bones,  etc.  The  changes  of  the  cerebrospinal  fluid,  finally,  are 
to  be  explained  only  by  the  assumption  of  a  chronic  spinal  meningitis, 
perhaps  starting  especially  from  the  posterior  roots. 

The  course  and  prognosis  of  tabes  dorsalis,  present  on  the  whole,  and  in 
spite  of  some  individual  differences,  a  very  sorry  spectacle.  The  preceding 
observations  show  the  usual  course.  The  patients  mostly  suffer  for  a  few 
years  from  lancinating  pains,  perhaps  also  from  slight  disturbances  of  the 
bladder.  With  sufficient  self-observation,  they  recognize  already  a  slight 
disturbance  of  sensation  in  the  feet,  some  uncertainty  in  walking  in  the  dark; 
the  examination  at  this  time  usually  discloses  pupillary  changes,  and  a  dif- 
ference in  or  absence  of  the  Achilles  and  patellar  reflexes,  possibly  also  some 
hyperesthesia.  Diplopia,  disturbances  in  the  muscles  of  the  eye,  impotence 
also  appear.  As  a  rule  years,  ten  or  more,  have  passed  since  the  first  "light- 
ning pains."  Then  are  added  increased  disturbances  of  sensation  and 
ataxia  in  the  legs,  gradually  also  in  the  arms,  which  robs  the  patients  more 
and  more  of  the  ability  to  move  (the  demarcation  of  a  third  stage  in  the 
symptomatic  picture  of  tabes,  the  paraplegic  stage,  mostly  resting  upon 
this,  I  consider  as  arbitrary) .  The  further  extension  of  the  process  to  the 
cortex  of  the  brain,  is  usually  less  clear,  since  some  phenomena  of  the  cerebral 
nerves  appear  much  earlier;  on  the  other  hand,  certain  cerebral  symptoms 
never  or  very  rarely  (athetosis)  supervene,  and  the  transition  to  paresis,  as 


266  DISEASES  OF  THE  SPINAL  CORD 

has  been  mentioned,  is  not  frequent  in  typical  tabes.  The  final  issue  is 
usually  fatal,  though  death  relatively  infrequently  is  due  to  direct  symptoms 
of  the  disease  (inanition  in  gastric  crises,  posticus  paralysis) ;  more  frequently 
it  is  due  to  intercurrent  diseases  (infective  diseases)  and  especially  to  second- 
ary consequence  of  tabetic  processes:  sepsis  due  to  trophic  disturbances, 
bed  sores  or  cystopyelonephritis,  as  the  result  of  external  infection  due  to 
the  sphincteric  lesions. 

There  are  several  characteristic  variations  from  this  usual  type.  In  the 
first  place,  the  course  may  be  very  much  more  rapid,  and  it  is  generally 
the  first  stage  which  is  very  much  shortened;  in  such  instances  the  first 
paroxysms  of  pain  may  be  followed  by  ataxia  in  a  few  weeks,  while  the  later 
stages  may  extend  over  the  usual  long  period  of  time.  Other  characteristic- 
ally atypical  forms  are  juvenile  tabes,  appearing  in  children,  almost  without 
exception,  upon  a  congenital  syphilitic  basis,  and,  as  a  rule,  of  a  relatively 
benign  character  (thus  far  only  a  single  autopsy  of  an  indubitable  case  has 
been  reported),  and  tabes  superior,  which,  on  the  other  hand,  is  a  very 
unfavorable  form  of  the  disease.  In  tabes  superior  the  symptoms  begin  in 
the  upper  extremities,  with  pain  in  the  ulnar  area  and  paresthesias,  also 
triceps  reflex  disturbance,  whereas  the  tendon  reflexes  in  the  legs  may  be 
retained;  in  such  cases  bulbar  symptoms,  dangerous  to  life,  usually 
supervene  very  early. 

In  addition  to  these  types,  authors  who  have  studied  a  large  number 
of  tabetic  patients,  like  Erb  and  Dejerine,  have  distinguished  additional 
clinical  types,  according  to  the  predominance  of  one  or  the  other  symptom: 
tabes  dolorosa  with  especially  violent  pains;  tabes  visceralis  with  violent 
crises  and  without  specially  marked  subjective  concomitant  symptoms. 
Even  though  the  value  of  this  classification  can  be  practical  only  in  so  far, 
as  such  cases  usually  preserve  their  characteristic  form  for  the  entire  and 
usually  protracted  course  of  the  disease,  for  one  form  the  predominance  of 
one  symptom  is  so  significant,  that  a  particular  anatomic  basis  has  long  been 
looked  for:  tabes  with  early  optic  atrophy.  According  to  the  investigations, 
especially  of  the  school  of  Pierre  Marie  (Leri  and  others)  25%  of  tabetics 
lose  their  sight  in  this  way;  in  these  cases  the  rest  of  the  nervous  system, 
frequently  participates  with  cerebral  symptoms,  but  rarely  with  spinal 
symptoms  of  marked  gravity  (with  the  exception  of  a  regular  loss  of  the 
tendon  reflexes);  the  further  course  also,  usually  protracted,  corresponds 
to  this  picture  so  that  a  few  authors  interpret  "tabetic  amaurosis"  and  the 
usual  form  of  tabes  as  two  different  localizations  of  the  same  process. 
Finally  to  the  deviating  forms  of  tabes,  there  belong  also  the  cases  of  imper- 
fectly developed,  rudimentary  tabes,  of  which  much  has  been  published  in 
recent  years.  The  conception  is  not  easily  defined;  as  a  matter  of  fact,  one 
can  speak  of  rudimentary  or  "abortive"  tabes  (Mobius)  only  when  the  con- 
dition, once  discovered,  persists  stationary  throughout  or  at  least  remains 


COURSE  AND  PROGNOSIS  OF  TABES  267 

thus  for  a  very  long  time,  and,  finally,  shows  only  few  symptoms  of  the 
disease.  The  possible  combinations  of  such  individual  symptoms  are,  as 
Erb  emphasizes,  naturally  very  numerous  and  varied,  but  in  almost  all  the 
cases,  there  is  found  one  of  three  symptoms:  loss  of  tendon  reflexes  (if  only 
of  one  single  Achilles  reflex);  pupillary  changes  (possibly  only  miosis 
without  loss  of  the  reaction  to  light);  lymphocytosis  of  the  cerebro-spinal 
fluid.  Naturally  in  such  a  patient,  whose  affection  is  recognized  acci- 
dentally, possibly  as  he  is  being  examined  by  the  physician  because  of 
"rheumatic  pains,"  one  cannot  tell  at  once,  whether  he  will  not  end  by 
becoming  afflicted  with  a  fully  developed  tabes,  but  not  rarely  this  is  not 
the  case,  and  we  may  then  assume  without  further  investigation,  that  it  is  a 
question  of  fixed,  quasi  "checked"  pathological  changes,  which  have 
possibly  affected  only  the  posterior  roots  and  meninges  and  did  not  go  any 
further  than  the  entrance  into  the  spinal  cord.  In  these  cases,  the  addition 
of  even  one  new  symptom  during  the  period  of  observation  is  usually  the 
signal  for  a  further  development  of  the  disease,  which  then  generally 
continues  steadily  to  progress.  Naturally,  this  rudimentary  tabes,  is  an 
especially  interesting  field  for  diagnosis. 

The  prognosis  of  tabes  is  apparent,  from  what  has  been  already  said. 
It  is,  quoad  sanationem,  bad.  Cases  of  genuine  recovery,  i.  e.,  of  complete 
disappearance  of  the  subjective,  and  a  total  or  almost  total  disappearance 
of  the  objective  symptoms  (areflexia  of  the  pupils  or  some  similar  symptom 
alone  remaining),  as  shown  in  the  pathological  changes  brought  to  light  by 
post-mortem  investigations,  are  exceedingly  rare,  but  do  occur  occasionally. 
Even  the  disappearance  of  single  symptoms  is  rare;  in  regard  to  this  v. 
Malaise  (from  Oppenheim's  polyclinic)  recently  reported  the  following: 
The  lancinating  pains  improved  in  37%  of  the  patients  (in  forms  that  ran 
a  wholly  favorable  course),  the  bladder  disturbances  in  8  to  10%,  the  sexual 
power  never,  when  once  disturbed  (but  here  one  must  consider  the  physio- 
logical decrease  that  normally  appears  with  advancing  years).  Very  fre- 
quently, on  the  other  hand,  the  crises  that  have  once  appeared,  disappear, 
that  is,  they  do  not  return,  whereas  atrophy  of  the  optic  nerve  nearly  always 
leads  to  total  blindness,  occasionally  only  after  ten  years,  on  an  average 
after  5  1/2  years.  Ataxia,  as  we  shall  see,  can  be  improved  by  therapeutic 
measures,  and  perhaps  even  spontaneous  improvement  is  possible;  in  regard 
to  the  paralysis  of  the  muscles  of  the  eyes  we  have  mentioned  already,  that 
even  in  their  intensity,  they  are  usually  subject  to  extraordinary  fluctuations. 

Cases,  in  which  the  so-called  late  symptoms  appear  early,  are,  as  in 
most  diseases,  prognostically  unfavorable  (ataxia,  trophic  disturbances); 
the  longest  possible  extension  of  the  first  stage  is  rightly  "considered  favorable. 
Frequently,  also,  the  disease  remains  stationary  for  years,  and  under  favor- 
able conditions  of  life,  the  patients  may  live  to  a  very  old  age,  so  that  accord- 
ing to  French  writers   (Bellugaud  and  Faure)   about  59%  of  all  tabetics 


268  DISEASES  OF  THE  SPINAL  CORD 

experience  an  "evolution  benigne,"  36%  an  "evolution  grave,"  and  5% 
recover.  I  consider  these  statistics  too  favorable,  and  should  prefer  to 
subscribe  to  the  statement  of  v.  Malaise,  who  out  of  70  patients,  found  2 
completely  freed  of  their  troubles,  26  retaining  their  working  capacity  for  a 
long  time,  and  in  good  general  condition,  30  with  constant  but  slow  pro- 
gression and  18  running  a  rapid  and  unfavorable  course.  Some  factors 
mentioned  when  discussing  the  aetiology,  influence  also  the  further  course 
of  the  disease.  Especially  unfavorable  results  come  from  alcohol,  sexual 
excesses,  traumatisms  and  physical  over-exertion,  extreme  degrees  of  tem- 
perature, persisting  cares  and  excitements. 

Somewhat  more  favorable  is  the  question  of  prognosis  quoad  vitam. 
Pierre  Marie  assumes  that  the  length  of  life  is  not  shortened  by  tabes  (51.5% 
died  after  the  sixtieth  year)  and  even  with  the  less  favorable  material  of 
Oppenheim,  the  statement  that  the  majority  of  the  patients  die  after  the 
sixtieth  year,  holds  good. 

The  diagnosis  of  tabes,  under  ordinary  circumstances,  is  easy  for  every- 
one who  understands  how  to  make  a  neurological  examination.  The  un- 
fortunately still  very  frequently  occurring  errors  in  the  diagnosis  of  the 
disease  are  to  be  explained  only  by  a  lack  of  such  knowledge.  The  above 
mentioned  abortive  cases  and  occasional  cases  with  peculiarly  complicated 
symptoms  or  course,  are  the  only  ones  which  may  cause  the  neurologist  dif- 
ficulty. We  have  mentioned  several  times  already  the  cardinal  symptoms, 
of  which  one,  at  least,  is  to  be  found  also  in  the  abortive  tabes,  the  "tabes 
fruste  " :  disturbance  of  the  tendon  reflex,  pupillary  disturbance,  lymphocytosis 
of  the  cerebro-spinal  fluid.  If  all  three  are  absent,  we  may,  with  a  consid- 
erable degree  of  certainty,  exclude  tabes;  at  the  most,  patients  with  per- 
sistent pains,  like  the  lancinating  ones,  should  always  arouse  a  suspicion 
of  tabes,  which,  however,  would  have  to  be  fully  confirmed  sooner  or  later 
by  the  appearance  of  one  of  the  symptoms  named.  Each  of  these  cardi- 
nal symptoms,  that  may  accidentally  be  found  in  a  patient,  must,  in  itself, 
suggest  the  possibility  of  the  existence  of  tabes;  if,  in  the  anamnesis  syphilis 
appears,  or  if,  in  the  history  of  the  existing  disease,  possibly  a  paralysis  of 
the  muscles  of  the  eye  is  spoken  of,  or  if,  in  the  objective  examination, 
hyperesthesia  to  cold  in  the  trunk  is  found,  the  thought  is  so  much  more 
reenforced.  Naturally  some  of  these  phenomena  appear  also  in  other 
diseases,  with  the  exception,  perhaps,  of  the  hyperesthesia  to  cold;  at  any 
rate,  the  combination  of  some  such  symptoms,  as  well  as  the  further  course 
of  the  disease,  usually  justify  the  diagnosis  of  tabes. 

Of  the  abortive  cases  we  have  already  spoken.  If  we  see,  for  instance,  a 
patient  with  slight  pupillary  differences,  lancinating  pains,  and  owning  to  a 
history  of  syphilis,  I  recommend  always,  beside  the  most  careful  examination 
as  to  sensation  and  tendon  reflexes,  above  all  to  perform  a  lumbar  puncture 
for  purposes  of  diagnosis;  if  this  turns  out  negatively,  I  am  inclined  to  exclude 


DIAGNOSIS  OF  TABES  269 

tabes  or  at  least  consider  it  most  improbable;  a  positive  result  in  lumbar 
puncture  makes  the  diagnosis  of  a  metasyphilitic  disease  of  the  central  ner- 
vous system  (according  to  its  frequency,  probably  a  tabes)  almost  certain,  in 
so  far  as,  and  this  is  easy  for  the  most  part  to  determine,  another  meningeal 
process  can  be  excluded.  I  should  arrange  the  diagnostic  symptoms,  in  the 
order  of  descending  importance,  as  follows: 

1.  Loss  of  the  tendon  reflexes. 

Miosis  and  reflex  rigidity  of  the  pupils  to  light. 
Lymphocytosis  of  the  cerebro-spinal  fluid. 

2.  Hyperaesthesia  to  cold. 

Other    sensory    disturbances  of    tabetic    type,  especially,  however, 
summation  and  retardation  in  conduction  of  pain. 
Anamnesis:  lancinating  pains;  former  syphilis. 

3.  Paralyses  of  the  muscles  of  the  eye. 

4.  Romberg's  symptom. 
Ataxia. 

Hypotonia  of  the  musculature. 
Sphincter  disturbances,  impotence. 

5.  Optic  atrophy. 
Crises. 

Trophic  disturbances. 

For  the  great  variety  of  symptoms  to  produce  diagnostic  difficulties,  is  a 
relatively  rare  occurrence,  and  happens  almost  only  when  totally  foreign 
phenomena  are  present  in  addition  to  the  complete  picture  of  tabes;  for 
instance,  hypertension  of  the  muscles,  exaggerated  tendon  reflexes,  periph- 
eral paralyses.  As  a  rule,  then,  it  will  be  a  question  of  combination  with 
other  diseases— sclerosis  of  the  lateral  columns,  peripheral  neuritis — only 
exceptionally  of  a  pure  tabes  with  an  atypical  course. 

Excluding  the  combinations  we  have  just  mentioned,  especially  the  so- 
called  combined  systemic  diseases,  which  vary  in  their  symptoms  according 
to  the  greater  participation  of  the  posterior  or  the  lateral  columns,  only  the 
various  forms  of  neuritis  are  of  differential  diagnostic  importance,  if  we  except 
also  the  rare,  usually  epidemic  ergotin  poisoning  (ergotin  tabes),  the  symp- 
toms of  which  correspond  almost  exactly  with  those  of  real  tabes.  Of 
neuritic  processes,  alcoholic  neuritis  most  frequently  hampers  our  diagnosis, 
more  rarely  the  diphtheritic  and  other  toxic  forms  of  neuritis  (diabetes  melli- 
tus  with  neuritis) .  Whereas  in  these  patients,  the  loss  of  the  tendon  reflexes 
and  the  disturbances  in  sensation  may  remind  one  strongly  of  tabes,  the  motor 
paralyses,  and,  above  all,  the  promptness  of  the  pupillary  reaction,  lead 
usually  to  the  correct  diagnosis.  Multiple  sclerosis  and  syringomyelia  may 
also  upon  occasion,  enter  into  the  problem  of  the  differential  diagnosis. 

In  spite  of  the  bad  prognosis,  the  treatment  of  tabes,  corresponding  to  the 
chronic  character  of  the  disease,  is  even  to-day  uncommonly  diverse,  and, 


270  DISEASES  OF  THE  SPINAL  CORD 

let  us  add,  by  no  means  devoid  of  results  from  the  standpoint  of  alleviating 
the  suffering  of  the  tabetic. 

Treatment  must  be  aimed  at  two  points,  inasmuch  as  only  in  the  rarest 
cases,  is  there  hope  for  complete  recovery.  It  must  be  adapted  to  the 
existing  stage  of  the  disease,  and  to  the  degree  of  the  result  that  probably 
may  be  attained,  and  must  not,  by  the  violence  of  applications,  or  by 
meddlesomeness  physically  or  mentally  over-exert  and  fatigue  the  patient. 

Because  of  the  meta-syphilitic  nature  of  the  disease  the  first  thing  to  do, 
is  to  try  a  course  of  anti-syphilitic  treatment,  to  give  first  mercury  or  iodides. 
Owing  to  the  differing  views  of  various  physicians  and  "schools"  this 
plunges  us  at  once  into  a  region  of  dispute.  There  is  comparative  agree- 
ment as  to  the  fact,  that  in  particular  mercurial  treatment  does  as  a  rule  no 
harm,  and  in  some  few  cases  produces  a  slight,  but  definite  beneficial  effect. 
From  this  middle  point,  however,  the  positions  of  the  extreme  camps  deviate 
considerably  in  both  directions.  While  some  physicians  reject  the  Hg.  cure 
as  useless  and  occasionally  injurious,  Erb,  especially,  defends  it  earnestly; 
in  his  experience,  it  does  "absolutely  no  harm,"  is  well  tolerated,  and  has 
often  an  excellent  influence  especially  on  the  lighter  symptoms  of  the  disease 
(disturbances  in  sensation,  etc.).  I,  myself,  should  like  to  say  that  I  never 
saw  mercurial  treatment  make  the  condition  worse  (I  am  a  little  doubtful  only 
in  cases  of  patients  with  optic  atrophy,  though  I  can  not  confirm  the  harmful 
influences  of  the  treatment  described  by  some  authors).  Direct  favorable 
consequences,  which  could  be  ascribed  only  to  the  Hg.  cure,  I  have  seen  but 
rarely;  on  the  other  hand,  it  has  been  quite  the  usual  thing  in  my  cases,  that 
with  regular  treatment  by  inunction,  repeated  annually  or  every  other  year, 
the  patients  were  subjectively  in  good  condition,  were  able  to  perform  work, 
gained  weight,  and  remained  for  years  about  the  same.  Some  of  the  patients 
ascribed  considerable  improvement  to  the  inunctions  and  asked  for  them 
again  and  again.  Naturally,  even  mercury  fails  to  produce  results  in  a 
number  of  cases,  and  I  have  not  succeeded  definitely,  any  more  than  have 
most  of  the  French  authors,  in  discovering  in  the  lymphocytic  content  of  the 
spinal  fluid — which  denotes  the  condition  of  the  meninges— any  difference 
worthy  of  mention  before  and  after  the  treatment.  On  the  whole,  I  recom- 
mend mercurial  treatment  in  recent  cases  (it  constitutes,  in  my  opinion,  the 
only  prophylactic  method  worthy  of  recommendation,  after  syphilis  has  been 
acquired,  especially  for  neuropathic  individuals,  though  one  must  concede, 
that  even  after  syphilis  has  been  treated  with  the  most  painstaking  correct- 
ness, a  tabes  may  appear  later),  in  syphilis  still  active  in  association  with 
tabes,  and  in  general  in  those  cases  in  which,  after  definitely  or  probably 
diagnosed  syphilis,  no  mercurial  treatment  has  been  used  for  some  time. 
The  only  condition  is  that  of  moderate  vigor  of  the  patient  and  the  possibility 
of  control  of  the  treatment  by  the  physician.  If  syphilis  may  be  excluded 
with  probability,  or  many  courses  of  treatments  have  already  proved  ineffec- 


TREATMENT  OF  TABES  271 

tualj  I  forego  mercury  as  a  rule.  Concerning  the  method  of  application,  I 
have  like  Erb,  had  the  best  results  from  small,  frequently  repeated  inunctions 
(daily,  for  30  days,  1  to  2  5  mercurial  ointment) ;  the  usual  care  of  the  gums 
for  the  prevention  of  stomatitis  is  especially  important.  Concerning  the  use 
of  iodides,  there  is  less  difference  of  opinion;  in  addition  to  or  between  the 
Hg.  treatments,  I  administer  for  a  space  of  3  to  6  weeks,  iodide  of  potassium 
(15  to  30  grs.  pro  die),  also  iodipin  (subcutaneously)  or  saiodin  (20  to  30  grs. 
pro  die),  and  have  seen  from  this  treatment  only  favorable  results;  special 
consideration  must  be  given  the  appetite  and  digestion  of  the  patient,  which 
therefore  often  requires  that  internal  medication  be  replaced  by  subcutane- 
ous administration  of  iodipin,  which  otherwise  is  not  agreeable  to  either 
patient  or  physician.  Serological  syphilitic  therapy  unfortunately  has,  up  to 
the  present,  shown  no  effect  on  tabes.  Now  the  entire  host  of  nervina  follows ; 
in  order  to  make  a  change  here  and  there,  the  physician  must  have  at  his 
disposal  a  sufficient  supply  of  remedies.  Ergotin  (secale  cornutum  0.5  pro 
die)  I  have  used  but  seldom;  Charcot's  school,  however,  formerly  recom- 
mended it  warmly.  Argentum  nitr.  I  give  occasionally,  preferably  in  pills  (for 
instance:  Arg.  nitr.  15  grs.  strychni  grs.  ii.  in  100  pills,  one  daily  three  times), 
for  several  weeks,  in  combination  or  in  alternation  with  preparations  of 
strychnine,  of  which,  especially  in  the  form  of  Erb's  "tonic  pills"  I  have  seen 
excellent  results  (Ferr.  lactic.  25  grs.,  extr.  chin.  aq.  25  grs.,  Extr.  nuc.  vom. 
7  to  10  grs.,  extr.  gentian,  q.  s.  to  100  pills,  take  six  daily).  The  arsenical 
preparations  in  their  pure  form  I  do  not  like  very  much,  on  the  other  hand, 
I  can  recommend  warmly  the  cacodyl  preparations  (natr.  cacodyl.  0.02-0.1 
subcutaneously  pro  die)  from  my  own  experience,  for  continual  treatment. 
Of  atoxyl,  which  has  recently  been  used  as  an  anti-syphilitic  agent,  I  have 
seen  little  benefit  in  tabes.  Of  general  tonics,  one  must  mention  in  addition, 
and  for  occasional  trials,  glycerophosphate,  lecithin,  nucleogen,  thiodine,  re- 
cently inaugurated  by  the  French,  phytin,  etc.  The  same  purpose,  preserva- 
tion of  strength,  beside  the  nourishment  of  the  patient  is  served  by  the  host  of 
medicines  that  rouse  the  appetite,  and  the  artificial  food  preparations.  (Sal- 
varsan  is  of  no  benefit  in  tabes,  in  my  experience,  and  may  do  harm.     Burr.) 

As  an  attempt  at  local  treatment  at  the  place  of  the  disease,  we  may  name 
here  the  various  methods  of  influencing  the  spinal  cord  and  the  posterior 
roots.  In  the  front  rank  stands  the  galvanic  current,  which  may  be  used  in 
almost  every  stage  and  with  almost  every  patient. 

To  be  recommended  is  the  stabile  use  of  the  constant  current;  two 
equally  large,  medium  sized  electrodes  are  used,  one  on  the  nape  of  the  neck 
and  the  other  over  the  lumbar  enlargement,  using  a  current  of  8  to  10  milli- 
amperes,  alternately  3  to  4  minutes  in  each  direction,  without  brusque  inter- 
ruptions. Frequently  also  I  apply  a  larger  electrode  to  the  spine,  a  smaller  to 
an  extremity  (peroneus  point,  wrist)  or  use  Erb's  sympathetic  galvanization; 
the  small  cathode  over  the  cervical  sympathetic,  the  larger  anode  over  the 


272  DISEASES  OF  THE  SPINAL  CORD 

opposite  half  of  the  body, placed  in  three  "stations"  near  the  spinal  column, 
using  a  current  of  about  equal  strength.  Symptomatically,  also,  especially 
in  enteralgias  and  weakness  of  the  bladder,  the  galvanic  current  is  valuable : 
medium  sized  electrode  over  the  lumbar  segment  of  the  cord,  a  large  one 
over  the  abdomen  or  bladder.  I  use  the  faradic  current  (medium  strength) 
far  more  rarely,  actually  only  with  paresthesias  and  disturbances  in  sen- 
sation, therefore  more  symptomatically. 

As  local  application,  we  consider  also  counter-irritation  along  the  spinal 
column,  particularly  painting  it  with  tincture  of  iodine,  mercurial  plaster 
(combined  possibly  with  belladonna);  also  from  the  actual  cautery  (small 
point-size  thermo-cauterizations  on  and  near  the  spinal  column,  20  to  30  at 
every  sitting)  one  occasionally  sees  results,  especially  for  the  girdle  feeling, 
etc.  Furthermore,  I  wish  to  mention  here  the  various  methods  of  stretching 
the  spinal  cord,  or  the  spinal  column;  either  in  the  form  of  suspension  (it  is 
best  to  fasten  to  the  seated  patient  a  sort  of  Sayres  suspensory  apparatus  at 
nape,  chin,  possibly  also  with  shoulder  straps,  which  by  means  of  a  weighted 
rope  passing  over  a  pulley,  is  drawn  upward)  or  as  so-called  bloodless  stretch- 
ing of  the  nerves,  by  which — in  various  ways — the  knees  or  the  extended 
limbs  of  the  patient,  who  is  lying  on  his  back,  are  passively  brought  as  near 
as  possible  to  the  upper  part  of  the  body  and  kept  thus  fixed  for  some  time. 
The  hypotonia,  which  is  usually  present,  facilitates  this  method,  which,  on 
the  other  hand,  just  because  of  this  is  not  quite  devoid  of  risk.  The  stretch- 
ing procedures  are  praised  very  highly  by  a  large  number  of  writers.  The 
so-called  bloody  nerve-stretching  has  been  justly  abandoned. 

Massage  is  especially  valuable  for  bed-ridden  tabetics;  it  is  particularly 
indispensable  in  the  ataxic  stage,  partly  to  keep  the  musculature  in  good 
condition,  and  at  the  same  time  to  prepare  it  for  the  exercise  treatment,  to 
be  mentioned  below,  and  partly  as  an  aid  to  metabolic  processes  as  a  whole. 
To  watch  over  the  metabolism  and  the  state  of  nutrition  of  the  patient,  is  on 
the  whole  a  main  factor  in  the  therapy  of  tabes.  By  every  means,  moderate 
forced  feeding,  nourishing  foods,  methods  of  stimulating  the  appetite,  etc., 
the  physician  must  try  to  keep  the  body  in  such  condition,  that  it  is  capable 
of  action  and  resistance;  indubitably,  thus  alone,  greater  service  is  rendered 
the  individual,  but  especially  the  more  advanced  tabetic,  than  by  the  use  of 
complicated  medicinal  compounds. 

The  allotment  of  rest  and  exercise  is  of  great  importance.  Above  all,  the 
slightest  over-exertion  on  the  part  of  the  patient  must  be  avoided.  Accepting 
Edinger's  "using  up"  (exhaustion)  hypothesis,  it  would  be  a  good  thing  to 
force  the  patients  to  take  absolute  rest  for  some  time;  Edinger  himself  has 
recently  emphasized  this  again.  But  without  considering  the  fact,  that  in 
the  very  protracted  course  of  most  cases,  such  treatment  would  be  tantamount 
to  an  almost  unendurable  test  of  patience  on  the  part  of  the  sufferers,  accord- 
ing to  the  views  of  most  authors,  the  result  of  the  rest  cure  is  uncertain,  even 


TREATMENT  OF  TABES  273 

in  those  cases,  in  which  local  over-exertion  had  certainly  helped  to  bring 
about  the  disease  (isolated  ataxia  of  the  right  arm  in  tabetic  clerks). 

On  the  other  hand,  among  the  general  therapeutic  methods,  hydro-  and 
balneotherapy,  rank  next  in  importance  after  nourishment. 

Almost  all  methods  of  procedure  may  be  employed,  especially  in  the 
earlier  stages  of  the  disease;  they  may  be  used  for  a  long  time,  but,  on  the 
other  hand,  it  should  be  a  principle  never  to  employ  them  in  too  energetic 
a  form — especially  in  respect  to  the  temperature.  Few  things  can  injure  a 
tabetic  more  than  using  applications  too  extreme  in  cold  or  heat;  I,  myself, 
have  seen  several  tabetics,  who,  after  energetic  schematically  applied  Kneipp's 
cures,  showed  a  decided  turn  for  the  worse.  Among  the  water  "cures" 
that  can  be  used  at  home,  I  recommend  mostly  the  hip  baths  (as  a  rule, 
without  shower)  of  900  to  950  F.,  which  are  applicable  in  nearly  all  stages 
of  the  disease;  alternating  with  them,  or  as  a  separate  "cure"  one  can  give 
artificial  carbonic  acid  baths,  pine-needle  baths,  to  strong  patients  cold 
friction  baths,  to  the  weaker  ones,  lukewarm  douches. 

Not  only  upon  psychic  grounds,  but  especially  because  of  their  almost 
specific  effectiveness,  "bath  cures"  (watering  places)  are  to  be  recommended, 
among  which  must  be  named  first,  the  baths  of  Nauheim  and  Oeynhausen 
(carbonic  acid  thermo-baths) ;  here  too,  however,  baths  of  medium  intensity 
are  most  effective.  All  other  baths  are  inferior  to  these  (but  we  might 
recommend  in  addition  the  sulphur  baths — Aixla  Chapelle,  Neundorf — and 
the  indifferent  hot  baths — Baden-Baden,  Gastein,  Wildbad) ;  many  tabetics, 
however,  are  much  improved  by  simple  fresh  air  treatment  in  the  middle  and 
high  altitudes,  whereas,  sea  baths,  as  a  rule,  are  less  beneficial. 

The  great  variety  and  all  too  frequent  ineffectuality  of  our  therapy,  is 
shown  in  the  treatment  of  the  separate  tabetic  symptoms,  which  we  shall 
briefly  discuss. 

The  lancinating  pains  are  especially  difficult  to  combat;  as  the  attacks 
are  usually  short,  the  use  of  morphine  is,  as  a  rule,  to  be  dispensed  with, 
more  so  since  these  pains  generally  react  best  to  the  simple  analgesic  "  anti- 
rheumatic" agents:  aspirin,  pyramidon,  citrophen,  lactophenin,  especially 
antipyrin  and  antifebrin  in  the  form  of  Erb's  "mixed  powder"  with  codein 
(antipyrin  grs.  7,  antifebrin  grs.  5,  codein  gr.  1/3-gr.  1/4;  instead  of  anti- 
pyrin possibly  also  phenacetin),  migrainin,  etc.  If  these  agents  are  ordered 
in  large  doses,  or  in  short  intervals,  the  result  is  usually  favorable.  It  is 
more  difficult  to  combat  the  permanent  pains,  enteralgias,  girdle-pains, 
etc.,  and  most  difficult  of  all  the  genuine  tabetic  crises,  especially  the 
gastric  crises,  which  can  be  influenced  almost  only  by  large  subcutane- 
ous doses  of  morphine.  The  internal  medicines  above  mentioned  (and  in 
addition,  bismuth,  ansesthesin,  cerium  oxalate,  bromides,  etc.),  are  usually 
ineffectual;  I  have  occasionally  seen  results  from  lumbar  puncture  and 
from    the   injection    of   soda    nitrate    subcutaneously.     If   one   does   not 


274  DISEASES  OF  THE  SPINAL  CORD 

wish  to  give  morphine,  there  remains,  usually,  only  one  way,  namely, 
to  let  the  crises  spend  their  strength  and  to  attempt  to  keep  the  patient,  by 
complete  rest  and  artificial  nourishment,  on  a  high  plane  of  strength. 
Nor  are  the  external  applications  of  much  avail  here,  whereas  in  lancina- 
ting pains,  hydropathic  packs,  painting  with  iodine,  chloroform,  oil,  methyl- 
salicylate,  salves  and  balsams  may  occasionally  prove  useful.1 

The  other  sensory  disturbances  rarely  become  so  painful  or  (except  when 
trophic  changes  arise,  cf.  below),  so  dangerous,  that  they  need  a  special 
therapy;  labile  faradic  treatment,  farado-massage  may  be  used  for  par-  and 
anaesthesias. 

The  motor  disturbances  with  the  exception  of  ataxia,  likewise  need  no 
particular  treatment;  peripheral  paralyses  are  rare;  for  the  paralyses  of  the 
muscles  of  the  eye,  which  incline  towards  spontaneous  improvement, 
galvanization  may  be  tried. 

On  the  other  hand,  as  is  well  known,  the  treatment  of  ataxia  forms  one  of 
the  main  problems  of  modern  tabes  therapy,  in  the  form  of  what  is  generally 
called  the  Fraenkel  " exercise  therapy,"  which  was  variously  tested  and  com- 
pleted by  Fraenkel,  Goldscheider ,  Foerster,  and  others.  As  has  been  eluci- 
dated above,  we  understand  by  ataxia,  speaking  generally,  and  for  practical 
therapeutic  purposes,  a  disturbance  of  co-ordination,  of  the  co-operation  of 
the  individual  muscles  and  groups  of  muscles.  To  this  corresponds  the  single 
therapeutic  purpose:  restoration  of  the  lost  co-ordination  by  practising  this 
very  process.  Inasmuch  as  the  disturbance  evinces  itself,  according  to  our 
experience,  preponderantly  in  the  dark,  that  is,  betrays  itself  in  movements 
not  controlled  by  the  eye,  the  process,  in  practice,  is  very  simple;  the  move- 
ment that  has  been  disturbed  is  gradually  learned  again  (by  practice)  under 
the  control  of  the  eye,  until  it  eventually  succeeds,  even  without  observation. 
In  our  experience,  the  help  of  the  eyes  is  a  mighty  factor  in  the  movements  of 
ataxic  limbs,  and  allows,  even  without  special  practice,  many  movements  to 
be  performed,  that  would  be  wholly  impossible,  if  the  eyes  were  closed. 

The  exercise  therapy  is  to  affect  every  muscular  area  stricken  by  ataxia;  as 
a  rule,  of  course,  first  and  most  important,  the  legs.  Since  it  must  be  a  main 
principle,  that  the  patient  is  not  to  be  tired  out  (exhaustion  may  lead  to  out- 
spoken deterioration  in  the  condition  of  the  patient),  the  first  leg  practice 
should  take  place  in  bed;  from  the  most  simple,  there  should  be  gradual 
advance  to  the  difficult.  For  exercise  therapy  there  exists  a  long  line  of 
useful  directions  {Fraenkel,  Goldscheider  and  others)  which  can  be  used  in 
complicated  cases,  especially  also,  to  make  greater  variety  possible.  They 
describe  also  a  great  number  of  assisting  apparatus  (diagrams  to  be  followed 
out  by  hands  or  feet,  parallel  bars  to  walk  between,  etc.),  which,  however, 
in  the  majority  of  cases  can  be  dispensed  with,  especially  if  the  physician,  as 

1  Whether  the  results  obtained  by  internal  use  of  adrenal  preparations,  reported  by  Rohmer  to 
the  society  of  German  neurologists,  October,  1908,  are  confirmed,  has  not  yet  been  ascertained. 


TREATMENT  OF  TABES  275 

should  always  be  the  case,  watches  over  the  execution  of  the  exercises.  As  a 
rule  every  physician  will  be  able  himself  to  compose  easily  the  most  important 
movements.  Besides  the  gradual  learning  of  all  movements,  attention  must 
be  paid  above  all  to  the  exact  co-working  of  the  single  components  of  the 
most  essential  and  important  movements:  walking,  dressing,  undressing, 
eating,  writing,  etc.  Employing  the  necessary  patience,  the  method  leads  to 
improvement  in  nearly  every  case,  sometimes  to  an  almost  total  disappearance 
of  the  ataxia.  Of  course,  one  must  work  with  greatest  consideration  and  care 
for  weeks,  often  for  months,  in  the  beginning  several  times  daily,  for  a  few 
minutes.  They  are  really  contraindicated  only  during  the  first  weeks  of  an 
acute,  rapidly  appearing  and  increasing  ataxia  and  if  the  patient's  general 
state  of  health  is  in  too  low  a  condition.  During  other  intensive  treatments 
(energetic  bath  cures)  it  is  better  to  cease  the  exercise  therapy  temporarily/ 

The  other  symptomatic  indications  for  treatment  are  more  or  less  self- 
evident.  A  warning  must  be  sounded  against  medication,  directed  to  in- 
fluencing tabetic  impotence;  all  the  highly  recommended  remedies,  especially 
yohimbin,  the  effects  of  which  are  to  be  understood  as  a  stimulation  of  the 
sexual  sphere,  have  proved  to  be  unnecessary,  and  partly  injurious;  in 
lighter  cases,  rest,  a  sparing  therapy,  together  with. general  tonic  procedures, 
is  most  likely  to  produce  an  improvement,  which,  to  tell  the  truth,  is  usually 
transient.  We  can  not  give  sufficiently  strong  warning  against  all  extreme 
measures. 

The  bladder  disturbances  react  relatively  best  to  galvanization,  occasionally 
also  (bladder  weakness)  to  ergot  and  strychnine.  In  pronounced  retention, 
catheterization  is,  alas,  anavoidabh;  this,  after  longer  or  shorter  time,  as  a 
rule,  causes  an  infection  of  the  bladder,  which,  it  is  true,  can  be  treated  for 
some  time  by  washing  out,  etc.,  but  is  apt  finally  to  lead  to  ascending 
processes — pyelitis,  pyelonephritis.  In  tabetic  retentio  alvi,  medicines  are 
to  be  used  only  in  cases  of  extreme  necessity. 

The  trophic  disturbances  are  treated  according  to  general  surgical  rules; 
the  arthropathies  can,  as  a  rule,  be  treated  only  orthopsedically. 

Because  of  the  usual,  slowly  progressive  course  of  tabes,  a  somewhat 
typical  plan  of  treatment  may  be  outlined  for  many  cases.  To  a  large  extent, 
however,  this  plan  depends  upon  the  stage  in  which  the  disease  is  recognized. 
If  the  recognition  takes  place,  as  it  always  should,  at  the  beginning  of  the 
lancinating  pains,  for  which  the  majority  of  tabetics  consult  their  physicians, 
there  should  be  introduced,  under  the  above  (cf.  under  therapy)  conditions, 
a  mercurial  treatment,  best  by  inunction.  Naturally,  besides  this,  at  least 
during  the  recognizable  advance  of  the  disease,  every  active  mental  and 
physical  exertion,  especially  exciting  occupations,  sports,  hot  or  cold  baths, 
sexual  indulgence,  should  be  immediately  given  up,  or,  at  .least  curtailed. 
Mild  hydrotherapy  (hip  baths)  and  galvanization  can  even  now,  but  in  any 
case,  as  continuation  of  the  mercurial  treatment  lasting  possibly  four  weeks, 


2  76  DISEASES  OF  THE  SPINAL  CORD 

be  used  for  2  to  3  months.  In  cases  where  the  mercurial  treatment  is 
excluded  (cf .  above)  an  iodide  therapy  (iodide  of  potassium,  iodipin,  saiodin) 
is  mostly  to  be  recommended  as  a  substitute  for  2  to  3  months;  it  may 
be  used  also  along  with  the  Hg.  treatment,  or  as  its  continuation.  In  the 
second  place,  after  the  course  of  the  first  2  to  3  months,  a  generally  tonic 
method  may  be  employed  in  all  cases:  arsenic,  cacodyl,  ErVs  tonic  pills,  etc., 
for  4  to  8  weeks.  If  circumstances  permit,  a  "bath  cure"  should  be 
interpolated  in  the  summer,  at  Nauheim  or  Oeynhausen,  with  temporary 
galvanization. 

Then  in  the  normal  course,  a  pause  in  treatment  of  some  weeks  or  even 
months  is  to  be  recommended.  After  the  symptoms  have  come  to  a  stand- 
still, one  may  wait  to  the  end  of  the  first  year,  and  then  carry  out  a  similar 
program  in  the  following  years,  with  slight  modifications  (relative  to  hydro- 
therapy, the  use  of  tonics,  derivation  to  the  skin,  suspension).  If  the 
disease  makes  a  marked  advance,  the  therapy  must  naturally  keep  step 
with  it,  but  with  avoidance  of  every,  even  therapeutic  exertion,  for  the 
patient;  often  absolute  rest  is  now  best  for  the  patient.  Symptomatic 
measures  (especially  exercise  for  the  ataxia)  naturally  are  to  be  employed 
at  the  same  time.  In  the  last  stage  of  the  disease,  there  is  a  question  only 
of  care  and  palliative  measures,  not  infrequently  of  extensive  morphine 
therapy.  Even  now,  however,  mild  procedures,  as  galvanization,  may  be 
tried  with  some  success.  In  any  case,  so  long  as  no  serious  cachectic  com- 
plications (serious  pyelitis,  bed  sores)  are  present,  there  is  need  to  beware 
as  well  of  therapeutic  nihilism  as  of  meddlesomness,  since  in  spite  of  the 
slight  influence  we  are  able  to  exert  on  the  course  of  the  malady,  there  are 
few  diseases,  in  which  such  excessive  demands  may  be  made  upon  therapeutic 
resources  as  in  tabes  dorsalis. 

(b)  Friedreich's  Disease  (Hereditary  Spinal  Ataxia) 

BY 

S.  SCHOENBORN  (Heidelberg) 

Friedreich? s  disease  represents  only  a  subdivision,  though  the  best 
known  and  sharpest  defined,  of  a  larger  group  of  more  or  less  systematized 
diseases  of  the  central  nervous  system,  which  are  of  a  degenerative  nature, 
usually  show  hereditary  or  familial  propagation,  and  are  founded  in  part  at 
least  upon  an  anomaly  of  predisposition  of  the  central  nervous  system,  and 
develop  from  this.  Common  to  them  all,  is  a  more  or  less  well  developed 
degree  of  ataxia,  which  has  given  the  group  its  name.  To  this  are'  added, 
according  to  the  individual  form,  disturbances  of  reflexes,  scoliosis,  bulbar 
symptoms,  optic  nerve  changes,  and  disturbances  in  intellect.  The  best 
method  of  dividing  the  entire  group  may  be  that  recently  proposed  by 


FRIEDREICH'S  ATAXIA  277 

Raymond,  which  divides  it  into  4  separate  types  of  disease,  of  which  we 
must  recognize  2  as  indubitably  correct. 

1.  Spinal  form  or  Friedreich's  disease  sensu  strictiori,  with  ataxia, 
nystagmus,  speech  disturbances,  absence  of  tendon  reflexes,  scoliosis, 
club  foot. 

2.  Cerebellar  form  or  heredoataxie  cerebelleuse  {Marie)  with  ataxia, 
optic  nerve  changes,  paralysis  of  the  muscles  of  the  eye,  exaggeration  of 
the  reflexes,  vertigo,  intellectual  disturbances. 

Between  these,  there  exist  doubtless  transitional  forms  as  well  as  the 
combination  of  1  and  2,  which  Raymond  classifies  as  an  independent  fourth 
group:  "type  generalise"  of  familial  character.  Whether  an  independent 
bulbar  form  (with  dyspnoea,  vomiting,  cardiac  arythmia)  exists,  seems  to 
me  doubtful  from  the  literature;  I  myself  have  seen  no  such  case.  Raymond 
defines  such  a  form  as  the  last  subdivision.  Let  us  now  turn  to  Friedreich? s 
disease,  in  its  stricter  sense. 

Friedreich's  disease  is  of  rare  occurrence.  In  1901  I  could  collect  from 
the  literature  only  a  few  more  than' 200  cases;  meanwhile  the  number  may 
at  the  most  have  doubled.  Apparently  the  disease  occurs  disproportionately 
less  frequently  in  Germany  than  in  England  and  North  America.  Of  its 
origin  we  know  but  little.  It  seems  certain  that  it  does  not  owe  its  develop- 
ment to  a  definite  poison,  differing  in  this  from  the  otherwise  so  similar 
tabes,  but  that  many  factors  may  serve  as  "agents  provocateurs."  Very 
often  the  disease  is  familial,  but  it  is  very  seldom  directly  hereditary.  This 
may  be  due  to  the  lack  of  possibility  of  transmitting  the  disease  by  heredity, 
since  it  is  already  far  advanced,  when  the  patients  arrive  at  marriageable  age, 
partly,  as  Bouche  has  it,  the  disease  itself  may  not  be  transferable,  but  only 
the  neuropathic  tendency  and  degeneration,  which  (because  of  alcoholism, 
tuberculosis,  syphilis)  is  actually  found  in  the  parents  of  the  patients.  It 
is  probable  that  a  slowly  progressing  inhibition  in  development  of  the 
spinal  cord,  which  quite  early  is  found  very  small,  is  the  primary  cause, 
which  gives  rise  to  the  disease  picture;  the  disease  may  then  be  excited  by 
infectious  diseases,  possibly  also  by  traumatism. 

The  disease  begins  very  early,  usually  between  the  6th  and  14th  years; 
all  cases  developing  after  the  25th  year  are  doubtful. 

The  pathologic-anatomical  conditions  are  rather  typical.  The 
spinal  cord  is  noticeably  attenuated,  especially  in  the  posterior  portions 
(occasionally,  also,  in  toto).  To  this  corresponds  an  ascending  degeneration 
of  the  posterior  columns,  always  of  the  columns  of  Goll,  occasionally 
partly,  of  the  columns  of  Burdach  also.  As  a  rule,  the  lateral  columns  are 
affected  also  and  most  frequently  the  lateral  cerebellar  tracts,  somewhat 
less  frequently,  the  lateral  pyramidal  columns.  The  columns  of  Clarke 
and  of  Gower  may  be  affected  too,  rather  exceptionally  the  anterior  pyramidal 
columns  also.     By  French  authors  (Dejerine)  emphasis  is  laid  on  the  fact 


278  DISEASES  OF  THE  SPINAL  CORD 

that  the  posterior  roots  and  the  peripheral  nerves  degenerate  as  well  (motor 
and  sensory),  which,  however,  is  described  as  inconstant  and  insignificant 
by  German  authors  (Oppenheim).  The  medulla  oblongata  and  the  cere- 
bellum, however,  do  not  show  any  changes  in  typical  cases.  The  degenera- 
tion affects  axis  cylinders  as  well  as  medullary  sheaths;  in  severe  cases  the 
axis  cylinders  in  the  columns  of  Goll  in  the  cervical  part  of  the  cord  disappear 
entirely. 

The  similarity  to  the  process  in  tabes  dorsalis,  is  quite  considerable,  if 
one  regards,  as  do  the  French  authors,  the  degeneration  of  the  posterior  roots 
as  a  regular  thing,  and  the  difference  concerns  particularly  the  lateral 
columns,  which  remain  intact  in  tabes.  But  it  is  far  more  difficult  to  dis- 
cover the  pathogenesis  of  the  disease.  It  is  as  yet  quite  doubtful  whether 
the  posterior  columns  are  affected  primarily,  and  everything  else  secondarily, 
or  if  the  disease  actually  ascends  from  the  periphery  through  the  posterior 
roots.  The  latter  could  be  accepted  only  with  the  aid  of  the  hypothesis 
(Dejerine),  that  Friedreich's  disease  consists  in  a  deficient  development  of 
certain  neurons,  together  with  insufficient  replacement  of  the  fibres  used 
up  in  the  course  of  time.  This  is  contradicted,  however,  by  the  regular, 
intactn'ess  of  the  ganglion  cells  in  the  anterior  horns  and  of  the  spinal  ganglia, 
whereas  in  the  columns  of  Clarke  the  ganglion  cells  are  often  found  destroyed. 
Also  the  Scherbak  hypothesis  of  primary  degeneration  exclusively  of  fibres 
passing  to  or  .from  the  cerebellum,  is  still  lacking  in  pathologic-anatomical 
proof,  however  significant  it  might  be  in  regard  to  the  above  mentioned  prin- 
ciple of  dividing  this  group  of  diseases.  The  pathogenesis  of  the  individual 
symptoms  we  shall  have  to  discuss  later.  On  the  whole,  then,  we  shall 
have  to  consider  Friedreich's  disease  in  the  pathologic-anatomical  sense  as  a 
combined  system  disease  with  preponderant  participation  of  the  posterior 
columns. 

The  clinical  picture  of  the  symptoms  generally  corresponds  to  this. 
Nearly  always  the  children  themselves,  or  members  of  the  family  first  notice 
the  appearance  of  the  ataxia.  Their  gait  becomes  uncertain,  with  legs 
.spread  wide  apart;  they  stamp  while  walking;  they  spill  the  liquids  they  are 
carrying,  and  begin  to  write  illegibly.  If  the  movements  are  tested  individ- 
ually, a  decided  ataxia  is  seen  to  exist  in  the  intentional  as  well  as  the  unin- 
tentional movements,  and  it  is  usually  equally  pronounced  in  all  the  extremi- 
ties at  an,  early  date;  as  a  rule,  the  head  also  is  attacked  in  the  form  of  the 
so-called  static  ataxia:  the  patients  sway  even  while  at  rest,  i.  e.,  in  sitting  or 
standing,  rock  constantly  to  and  fro  with  the  head  and  trunk,  like  a  tree 
blown  about  by  the  wind.  This  instability  affects  all  movements  simultane- 
ously, and  reminds  one  of  the  ataxia  of  tabetics.  But  doubtless  there  is  an 
admixture  of  another  component  (especially  perceptible  in  walking),  which 
reminds  one  of  cerebellar  unsteadiness.  Very  many  patients  exhibit  besides 
the  ataxia  of  the  single  movements,  a  wholly  unsteady  gait,  as  if  intoxicated; 


SYMPTOMATOLOGY  OF  FRIEDREICH'S  ATAXIA 


279 


when  walking  they  shove  themselves  forward,  while  they  attempt,  to  a  cer- 
tain extent,  to  steer  the  body,  which  is  usually  held  stiff.  Occasionally  there 
is  even  a  certain  degree  of  propulsion.  With  eyes  shut,  the  unsteadiness,  as  a 
rule,  increases,  as  in  tabes,  but  the  patients  virtually  never  fall  down.  On 
the  whole,  because  of  the  extremely  slow  development  of  the  disease,  the 
patients  learn  to  control  their  movements  in  part,  in  spite  of  the  ataxia. 
Thus  one  of  my  patients,  in  spite  of  a  high  degree  of  ataxia  in  the  hands,  was 


Fig.  61. — Form  of  foot  in  Friedreich's  disease. 

able  to  draw  very  beautifully  and  write  very  legibly,  both,  of  course,  very 
slowly. 

Beside  this  disturbance  in  movement,  there  appears  frequently,  especially 
in  the  muscles  of  the  head  and  face,  also  of  the  hands,  a  muscular  restlessness, 
which  recalls  chorea  (grimaces)  or  perhaps  more  often  athetosis. 

At  the  same  time  the  mere  physical  strength  of  the  patient  is  not  decreased 
to  any  extent,  though  usually  the  arms  and  legs  grow  somewhat  emaciated. 
But  local  atrophies  are  very  rare,  though  the  frequent  pes  varus  (varo- 
equinus)  seems  to  suggest  them  in  Friedreich's  disease.  As  a  matter  of  fact, 
the  planta  pedis  is  usually  very  thin  and  the  prominence  of  the  arch  of  the 


28o  DISEASES  OF  THE  SPINAL  CORD 

foot  and  of  the  tendon  of  the  extensor  hallucis  longus  makes  the  disease  pic- 
ture very  peculiar,  but  I  never  found  actual  atrophy  in  it  (which,  however, 
is  claimed  for  it  by  other  authors),  but  rather  an  increase  in  the  size  of  the 
extensor  hallucis  longus.  There  appears,  moreover,  corresponding  with  the 
prominence  of  this  tendon  and  with  the  dorsal  flexion  of  the  great  toe,  an 
occasional  phenomenon  in  the  hand  ("main  bote")  in  the  form  of  a  hyperex- 
tension  of  the  last  phalanges.  In  this  condition,  slight  muscular  hyperten- 
sion may  be  seen  to  exist  in  the  parts  affected,  which  otherwise  is,  how- 
ever, usually  absent  in  this  disease.  But,  on  the  other  hand,  a  considerable 
hypotonia  of  the  muscles  is  scarcely  ever  found.  To  explain  these  otherwise 
astonishing,  local  muscular  hypertensions,  one  must  assume  that  they  are 
the  consequence  of  continuous  cramp-like  attempts  at  equilibration,  in  which 
the  great  toe  plays  the  preponderant  part.  (The  explanation  of  Cestan,  that 
the  Babinski  reflex,  usually  present  in  the  disease  (cf .  below)  has,  so  to  say, 
become  permanent,  is  not  sound)  (Fig.  61). 

Of  other  deformities  we  must  mention  the  extraordinarily  frequent 
kyphosis,  or  kyphoscoliosis,  which  is  probably  only  a  secondary  phenomenon 
resulting  from  the  lack  of  muscular  balance. 

Cutaneous  sensibility  as  well  as  deep  sensibility,  are,  strange  to  say, 
retained  as  a  rule.  Only  isolated  instances  of  disturbances  have  been  re- 
ported in  the  literature;  thus  one  of  my  patients  made  the  characteristic 
statement,  that  when  swimming,  he  did  not  know  where  his  legs  were;  as  a 
matter  of  fact,  it  turned  out  in  the  examination  that  coarser  disturbances  of 
the  sense  of  position  were  not  perceivable.  Oppenheim  mentions  a  similar 
case.  Usually,  however,  both  objective  and  subjective  disturbances  of 
sensation  are  wholly  lacking.  Pains  also  are  scarcely  ever  complained  of. 
The  tendon  reflexes  are  absent'm  all  definitely  marked  cases.  Of  the  cutaneous 
reflexes,  the  abdominal  and  cremaster  reflexes  are,  as  a  rule,  active,  and  the 
plantar  reflexes  show  the  form  of  the  so-called  Babinski  reflex,  slow  dorsal 
flexion  especially  of  the  great  toe,  upon  irritation  of  the  planta  pedis. 

In  regard  to  the  cerebral  nerves,  one  need  only  say  that  usually  nystagmus 
is  found,  corresponding  to  intention  ataxia,  and  that  speech  becomes  uncer- 
tain early,  now  scanning,  now  explosive  and  above  all  it  is  distinctly  slower 
than  normal.  The  other  cerebral  nerves  are  normal  in  pure  cases,  especially 
those  of  the  muscles  of  the  eyes  and  the  optic  nerve.  Disturbances  of  the 
sympathetic  nervous  system  also  (vasomotor  disturbances,  sphincter  affec- 
tions, crises)  are  in  contradistinction  to  tabes,  as  rare  as  pronounced  trophic 
changes. 

The  psyche  remains  normal.  In  one  of  my  cases  epileptiform  convul- 
sions were  noted.  As  to  combinations  with  bulbar  symptoms,  cf.  below. 
Concerning  the  pathogenesis  of  the  individual  symptoms,  it  is  to  be  remarked, 
that  ataxia  in  its  spinal  components,  is  to  be  traced  back  to  the  process  in  the 
posterior  columns;  in  its  cerebellar  components,  to  the  degeneration  of  the 


THE  DIAGNOSIS  OF  FRIEDREICH'S  ATAXIA  281 

lateral  cerebellar  tracts.  Whether  the  nystagmus  is  to  be  assigned  to  a 
cerebellar  tract,  remains  doubtful;  for  the  speech  disturbance  also,  a  clear 
anatomical  foundation  is  lacking.  The  Babinski  reflex  we  must  probably 
ascribe  to  the  (slight!)  lesion  of  the  pyramidal  tracts.  It  is  very  striking, 
however,  that  notwithstanding  the  serious  lesions  of  the  posterior  columns,  no 
disturbances  of  sensation  are  present.  It  seems,  therefore,  that  the  columns 
of  Goll  are  not  absolutely  necessary  for  the  localization  of  peripheral  sensa- 
tions, and  can  even  be  replaced  for  their  transmission;  perhaps  there  is  some 
connection  here  with  the  fact  that  in  spinal  ataxia  the  short  fibres  are  re- 
tained, whereas  the  long  fibres,  contained  for  the  most  part  in  the  columns 
of  Goll  are  largely  destroyed  (Dejerine) . 

The  course  of  the  disease  is  very  chronic,  and  it  may  drag  on  for  20  to  40 
years.  The  symptoms  increase  slowly,  but  none  of  them  in  itself  is  apt  to 
bring  on  death,  which  is  usually  caused  only  by  an  intercurrent  disease. 

Correspondingly,  the  prognosis  quoad  vitam  is  not  unfavorable,  quoad 
sanationem  is  absolutely  bad. 

The  diagnosis  of  pure  cases  is  easy.  It  is  in  itself  easily  distinguished 
from  all  pure  system  diseases;  from  tabes,  additionally,  by  the  lack  of  all 
disturbances  in  sensation  and  the  absence  of  eye  symptoms.  Somewhat 
more  difficult  may  be  its  separation  from  multiple  sclerosis,  the  intention 
tremor  of  which  may  resemble  ataxia;  nystagmus,  speech  disturbance,  youth, 
and  often  but  slight  spastic  phenomena  occur  in  it  also.  But,  as  a  rule,  here 
too,  the  difficulties  are  solved  by  the  exact  recognition  of  ataxia. 

As  great  rarities  some  combined  system  diseases  have  been  described, 
which,  like  Friedreich's  disease,  presented  a  combination  of  symptoms  of 
the  posterior  and  lateral  columns,  and  in  their  course,  much  resembled  it; 
but,  as  a  rule,  the  spastic  phenomena  predominated  in  them,  and  the  ataxia 
was  slighter.     Naturally,  a  sharp' division  is  often  impossible  in  these  cases. 

On  the  other  hand,  we  have  still  to  consider  its  demarcation  from  the 
disease  picture  of  heredoataxie  cerebelleuse  (of  Pierre  Marie) . 

In  this  disease,  an  ataxia  develops  in  middle  age,  which  exhibits  an 
obvious  but  not  always  an  exclusive  cerebellar  character.  To  this  are  added 
paralyses  of  the  muscles  of  the  eye,  and  nearly  always  atrophy  of  the  optic 
nerve,  frequently  psychic  disturbances  (imbecility),  exaggerated  tendon 
reflexes.  On  the  other  hand,  we  miss  the  results  of  defects  in  muscular 
balance,  which  correspond  more  nearly  to  spinal  ataxia — namely,  talipes 
cavus,  and  scoliosis.  The  autopsy  disclosed  in  pure  cases  of  this  disease 
an  abnormally  small  cerebellum,  occasionally  also  some  diminution  in  the 
entire  central  nervous  system,  degeneration  of  the  spinal  tracts  which  stand 
in  connection  with  the  cerebellum  and  of  the  posterior  columns.  But  these 
pure  cases  (also  described  by  Marie  von  Londe,  Miura,  Switalski,  and  others) 
are  still  more  rare  than  pure  cases  of  hereditary  spinal  ataxia.  On  the  other 
hand,  in  recent  years,  observations  have  been  accumulating,  in  which  symp- 


282  DISEASES  OF  THE  SPINAL  CORD 

toms  of  both  diseases  are  combined:  Friedreich' 's  disease  with  psychic  symp- 
toms, optic  atrophy,  Marie's  disease  with  spinal  ataxia,  kyphoscoliosis, 
talipes  cavus.  Even  in  one  and  the  same  family  Friedreich's  disease  has 
been  observed  in  pure  and  in  mixed  form  {Raymond).  Still  rarer  are  the 
symptoms,  which  (cf.  above)  may  be  classified  under  the  special  group  of 
the  "bulbar  form";  disturbances  in  respiration,  in  so  far  as  they  do  not  come 
from  ataxia  of  the  respiratory  muscles,  usually  in  the  form  of  dyspnoea; 
cardiac  disturbances,  recalling  myocarditis  (which  according  to  French 
authors,  Lannois  and  Porot,  frequently  occur  in  the  pure  Friedreich  as  well), 
vomiting.  That  these  symptoms  occur  when  the  disease  process  is  continued 
to  the  medulla  oblongata,  is  indubitably  true;  the  formulation,  however,  of  a 
"beredoataxiebulbaire,"  seems  nevertheless  artificial. 

Therapy,  unfortunately,  offers  little,  but  luckily,  with  one  exception, 
there  is  but  little  pressing  need  of  it  in  Friedreich's  disease.  This  exception 
concerns  the  removal  of  the  ataxia,  which,  if  it  can  not  be  entirely  cured,  can 
at  least  be  vigorously  combated  by  practice  of  the  most  important  muscular 
movements  in  the  form  of  FraenkeVs  exercises.  Very  many  patients,  because 
of  the  gradual  development  of  the  disease,  succeed  by  themselves,  in  exten- 
sively exercising  the  groups  of  muscles  most  necessary  to  their  life.  (For 
more  details  on  FraenkeVs  exercises,  cf.  under  the  article  "tabes".)  After 
all,  therapy,  except  for  a  certain  prophylaxis  especially  in  infectious  diseases 
in  families  of  a  neuropathic  diathesis  or  in  those  already  affected  by  Fried- 
reich's disease,  can  proceed  only  symptomatically.  In  longer  treatment, 
the  same  rules  hold  good  as  for  the  "tonic  procedure"  in  tabes. 

(c)  Spastic  Spinal  Paralysis.1     (Primary  Lateral  Sclerosis) 

BY 

FR.  JAMIN  (Erlangen) 

The  disease  picture  known  as  spastic  spinal  paralysis,  the  clinical  expres- 
sion of  a  purely  motor  system  disease,  a  primary  degeneration  of  the  py- 
ramidal tracts,  is  but  rarely  observed.  It  seems,  however,  that  functionally 
the  pyramidal  tracts  are  very  easily  affected,  like  the  posterior  columns  of  the 
spinal  cord,  so  that  corresponding  to  their  functional  or  anatomic  injury, 
the  symptom  complex  of  spastic  spinal  paralysis,  or  of  spastic  paraparesis, 
temporarily  or  preponderantly  steps  into  the  foreground  in  many  localized 
or  diffuse  diseased  conditions  of  the  spinal  cord.  But  even  though  it  seemed 
for  a  long  time  as  if,  according  to  anatomic  findings,  the  sharply  drawn 
clinical  picture  of  the  disease  described  by  Erb  in  1875,  following  up  Charcot's 
hint,  could  not  be  characterized  as  a  pure  system  disease,  nevertheless  in  more 
recent  times  a  series  of  clinically  as  well  as  anatomically  thoroughly  investi- 

1  Infantile  spastic  paraparesis  (Little's  and  related  diseases)  is  treated  in  the^chapter  entitled 
"Organic  nervous  diseases  of  childhood"  by  J.  Ibrahim. 


SYMPTOMATOLOGY  OF  SPASTIC  SPINAL  PARALYSIS 


283 


gated  cases  have  proven  that  there  is  actually  a  primary  motor  system  disease 
of  the  spinal  cord,  confined  to  the  pyramidal  tracts,  with  the  symptoms  of 
spastic  paralysis,  and  thereby  not  only  a  symptomatological,  but  also  a 
nosological  individual  place  is  secured  for  spastic  spinal  paralysis. 

Symptomatology. — The  spastic  spinal  paralysis  of  adults  is,  in  its 
purely  systemic  form,  a  very  protracted  disease  (extending  for  years  and  dec- 
ades after  the  onset  of  the  first  phenomena),  which  usually  occurs  in  the  2nd 
to  4th  decade  of  life,  progressing  very  gradually,  but  most  constantly,  and 
characterized  by  but  few  subjective  disturbances.  As  a  rule,  the  first  phenom- 
ena appear  in  the  lower  extremities. 
The  patients  notice,  when  walking, 
that  they  become  tired  easily,  and 
that  a  certain  heaviness  and  stiffness 
in  the  legs  interferes  with  the  more 
active  movements,  especially  such  as 
running  and  jumping.  Should  their 
vocation  make  greater  demands  upon 
them,  unpleasant  sensations  of 
weariness  appear,  occasionally,  too, 
a  feeling  of  cold  .in  the  legs  (they 
are  more  than  ordinarily  difficult  to 
move),  but  violent  pains  or  pares- 
thesias do  not  occur.  As  the  dis- 
ease advances,  the  patients  become 
more  and  more  helpless;  they  can 
move  forward  only  by  means  of 
small,  dragging  steps,  and  become 
so  stiff  in  the  legs,  that  when  they 
meet  obstacles,  they  are  no  longer 
able  to  avoid  them  quickly,  therefore 

they  stumble  easily,  and,  occasionally,  fall.  A  number  of  the  patients  can, 
with  the  aid  of  canes,  preserve  for  an  extraordinary  length  of  time  a  limited 
walking  ability;  others,  on  the  other  hand,  because  of  the  increasing  stiff- 
ness and  weakness  of  the  legs,  and  the  supervening  contractures,  finally 
become  bedridden. 

Examination  enables  us  to  recognize  quite  early  the  spastic-par etic 
symptom  complex. 

Particularly  striking  is  the  stiffness  of  the  legs,  the  hypertonia  of  the 
muscles,  which  is  exhibited  even  in  conditions  of  rest,  but  more  in  active  and 
more  especially  in  passive  attempts  at  movement.  It  leads  to  a  functional 
stiffening  of  both  legs,  in  the  joints  of  the  hip,  knee,  and  foot,  which  occasion- 
ally is  more  marked  at  the  beginning,  on  one  side,  than  on  the  other.  The 
stiffness  prevails  in  the  extensors,  the  gluteal  muscles,  the  adductors,  the  power- 


FiG.  62. — Spastic  spinal  paralysis;  posture  in 
sitting.     {After  Schoenborn  and  Krieger.) 


284  DISEASES  OF  TEE  SPINAL  CORD 

ful  mass  of  the  quadriceps  muscles,  and  in  the  musculature  of  the  calves  of  the 
legs.  In  advanced  cases,  therefore,  the  patients  can  not  flex  their  legs 
sufficiently  at  the  hips  and  the  knees,  to  be  able  to  sit  down  (Fig.  62).  When 
trying  to  sit,  the  patient  leans  against  the  edge  of  the  chair,  almost  as  stiff  as  a 
stick,  while  the  legs,  almost  at  full  extension  at  the  knee,  remain  floating  in  the 
air.  It  is  only  with  great  effort  and  with  the  use  of  considerable  strength, 
that  passive  flexion  in  the  hip-  or  knee-joint  may  be  carried  out.  Quickly 
executed  attempts  at  passive  movements  merely  increase  the  muscular 
resistance.  In  the  ankle-joint,  the  stiffness  may  progress  to  such  an  extent, 
that,  as  a  result  of  the  "muscular  joint  ankylosis"  (v.  Strilmpell) ,  active  and 
passive  movements  of  the  foot  become  virtually  impossible.  Frequently  the 
extension  of  the  leg  as  a  whole  is  so  forcefully  produced,  so  fixed  by  muscu- 
lar tension,  that  the  patient  can  be  lifted  from  a  supine  position,  by  one  leg, 
almost  like  a  board.  Even  in  passive  lateral  movements  of  one  leg,  the  pel- 
vis and  the  other  leg  follow,  whereas  with  careful  yet  forceful  overcoming  of 
the  muscular  tension,  it  may  be  demonstrated  that  the  possibility  of  move- 
ment is  retained  in  the  apparatus  of  the  joints  and  ligaments. 

The  fact  that  the  patient,  in  spite  of  such  an  increase  of  the  functional 
obstacles,  is  still  able  to  walk  for  considerable  distances  in  an  upright  posi- 
tion, shows  that  no  decided  paralysis  of  the  legs  is  present.  As  a  matter  of 
fact,  in  movements  of  resistance,  the  muscles  of  the  leg,  and  especially  the 
extensors,  prove,  very  frequently,  to  be  still  powerful,  and  we  must  ascribe 
preponderantly  to  the  increased  muscular  tension,  the  fact  that  the  active 
movements  are  so  troublesome,  so  hampered  and  slow.  Nevertheless,  in 
many  cases,  a  diminution  of  the  actual  strength  of  the  legs  is  also  demonstrable, 
independent  of  the  obstacle  of  tension,  especially  in  the  abductors  and 
flexors  in  the  hip-joint,  the  flexors  in  the  thigh  (biceps,  semimembranosus 
and  semitendinosus)  and  in  the  dorsal  flexors  of  the  foot.  This  paresis  of  the 
flexors,  i.  e.,  the  shorteners  of  the  leg,  combined  with  actually  greater  strength 
in  the  extensors  or  lengtheners  of  the  leg,  and  the  hypertonia  prevailing  in 
the  latter,  influences  the  posture  and  the  gait  in  spastic  paraparesis  character- 
istically: the  patients  in  order  not  to  fall  backwards,  because  of  the  extension 
of  the  hip,  move  the  weight  of  the  body  far  forwards,  the  outstretched  hands 
at  the  same  time  seeking  new  points  of  support  with  the  aid  of  canes  or  by 
grasping  furniture  (Fig.  63).  Both  legs  remain  in  full  extension,  the  feet  in 
plantar  flexion;  and  frequently  the  patients  step  only  on  the  balls  of  the  toes, 
until  by  standing  longer,  the  weight  of  the  body  overcomes  the  tension  of 
the  muscles  of  the  calf  of  the  leg.  The  thighs  are  adducted,  the  feet 
turned  slightly  inward.  In  walking,  the  legs  can  scarcely  be  raised  or 
flexed  at  the  knee;  in  almost  full  extension,  one  after  the  other  is  pulled 
forward  laboriously  in  a  short  circle,  the  tips  of  the  toes  are  allowed  to  drag, 
or  trailed  forward  and  thus  a  short  step  is  taken.  At  the  same  time, 
because  of  the  overbalance  of  the  adductors,  the  knees  and  the  balls  of 


SYMPTOMATOLOGY  OF  SPASTIC  SPINAL  PARALYSIS        285 

the  toes  frequently  rub  against  each  other  on  the  medial  side.  Because 
of  this  the  shoes  are  worn  off,  especially  in  the  foremost  part  of  the  sole, 
and  on  the  inner  side  of  the  tips  of  the  toes.  On  sandy  ground,  slightly 
curved,  almost  parallel  lines  show  the  tracks  of  the  short,  dragging  steps. 
And  since  walking  is  accomplished  rather  by  dragging  the  extended  leg 
along,  the  upper  part  of  the  body  being  bent  forward,  than  by  means  of  an 
elastic  lifting  swing,  and  replacement  of  the  leg,  as  is  performed  by  healthy 
individuals,  it  produces  a  characteristic  rhythmic  scraping  noise  in  short 
intervals. 

Next  to  the  phenomena  of  hypertonia  and  paresis  in  certain  muscle 
groups,  the  tendency  to  associated  movements  is  characteristic  of  the  spastic 
changes.  Their  common  foundation  lies  probably  in  the  fact  that  the 
movements  of  the  legs,  interfered  with  by  weakness  and  increased  muscular 
tension,  are  synergetically  possible  only  by  the  co-operation  of  larger  groups 
of  muscles,  trained  to  perform  primitive  movement  complexes,  whereas  the 
volitional  suppression  of  these  combinations  of  movements,  in  favor  of  finer, 
isolated  movements,  which  were  learned  during  the  course  of  ,life,  though 
possible  to  the  normal  man,  is  now  impossible.  Especially  clear  does  this 
disturbance  appear  in  spastic  individuals  in  the  movements  of  the  foot  and 
toes.  Whereas,  in  many  cases,  an  isolated  voluntary  movement  of  the 
great  toe,  or  of  the  entire  foot,  e.  g.,  dorsal  flexion  can  no  longer  be  performed 
by  the  patient,  strong  active  flexion  of  the  limb  at  the  hip  or  the  knee  is  seen 
to  be  accompanied  by  a  strong,  insuppressible  dorsal  flexion  .of  the  foot, 
with  conspicuous  protrusion  of  the  tendon  of  the  tibialis  anticus,  {StriimpelV  s 
tibial  phenomenon).  In  lifting  the  whole  leg,  often  also  during  attemps  at 
walking,  a  very  marked  dorsal  flexion  of  the  great  toe  appears  {StriimpelV s 
toe  phenomenon) ,  which  can  not  be  produced  isolated  to  the  same  excessive 
degree  by  active  effort.  Flexion  of  the  leg  at  the  hip,  which  is  almost  im- 
possible when  the  leg  is  in  extension,  is  also  far  more  easily  accomplished 
against  resistance  if  at  the  same  time  the  leg  proper  be  flexed  and  the  foot 
put  in  dorsal  flexion,  and,  on  the  other  hand,  extension  of  the  hip  is  difficult 
without  simultaneous  extension  of  the  knee  and  plantar  flexion  of  the  foot. 

The  permanent  hypertension  and  excessive  excitability  in  individual 
muscles  or  groups  of  muscles,  not  only  during  voluntary  functional  activity 
but  also  to  reflex  stimuli,  leads  usually  to  a  slight  change  in  the  permanent 
position  of  the  lower  extremities,  to  contractures.  Usually  the  extended 
position  of  hip  and  knee  is  accompanied  by  plantar  flexion  with  slight  inward 
rotation  and  supination  of  the  foot  and  dorsal  flexion  of  the  great  toe,  with 
moderate  adduction  of  the  thigh.  In  other  cases,  there  appear,  especially 
in  long  persisting  affections,  flexion  contractures  in  the  hip-  and  knee-joints 
in  spite  of  the  continuation  of  the  equino-varus  position  of  the  feet  and  the 
prominence  of  the  tendon  of  the  long  extensor  of  the  great  toe. 

Without  exception  spastic  spinal  paralysis  is  accompanied  by  a  marked 


286  DISEASES  OF  THE  SPINAL  CORD 

exaggeration  of  the  tendon  and  periosteal  reflexes  in  the  lower  extremities. 
Occasionally  it  is  difficult  to  demonstrate  this  exaggeration  of  the  reflex 
because  of  the  enormous  muscle  tension,  but  even  then  it  is  usually  possible 
with  the  limbs  in  a  suitable  position  of  rest;  the  patellar  and  a  chilles  tendon 
reflexes  may  be  elicited;  marked  patellar  and  ankle  clonus  is  very  frequently 


Fig.  63. — Spastic  spinal  paralysis;  posture  in  standing.     (Erlangen  Medical  Clinic.) 

also  induced  in  this  condition.  The  reflexogenous  zones  are  considerably 
enlarged;  the  reflex  movements  may  occur  on  the  other  side  as  well,  and  in 
muscles  at  some  distance.  Xot  rarely,  under  the  influence  of  weight  alone, 
when  the  legs  are  hanging  down,  or  after  trifling  spontaneous  and  reflex 


SYMPTOMATOLOGY  OF  SPASTIC  SPINAL  PARALYSIS        287 

movements,  one  leg,  or  even  both,  are  thrown  for  some  little  time  into  clonic 
tremulous  reflex  movements,  which,  like  genuine  reflex  spasms,  can  be  sup- 
pressed only  with  considerable  trouble  by  fixation  of  the  limb  for  some  time, 
or  by  vigorous  stimulation  of  the  skin.  These  spasms  become  very  annoying 
to  the  patient.  In  standing  and  walking,  also,  there  appears  often,  as  a 
consequence  of  the  abnormally  vigorous  reflex  excitability  of  the  muscles  of 
the  calf  of  the  leg,  and  the  peculiar  position  of  the  toes,  a  reflexly  conditioned 
quivering  and  trembling  over  the  entire  body.  Upon  tapping  the  side  of  the 
dorsum  of  the  foot,  there  frequently  appears,  instead  of  the  normal  dorsal 
flexion,  plantar  flexion  of  the  toes  (K.  Mendel). 

Of  the  cutaneous  reflexes,  those  of  the  abdomen  and  the  cremaster-reflex 
occasionally,  appear  to  be  diminished,  but  usually  suffer  no  change  worth 
mentioning.  The  cutaneous  reflexes  in  the  legs,  especially  those  of  the 
sole  of  the  foot,  are,  as  a  rule,  exaggerated  and  changed  in  a  typical  manner : 
lively  flexing  movements  of  the  entire  limb,  or  manifold  combined  reflex 
muscular  contractions  follow  upon  energetic  stimulations  of  the  skin. 
Especially  characteristic  is  the  Babinski  sign,  slow  dorsal  flexion  of  the  great 
toe,  upon  irritation  of  the  sole  of  the  foot.  This  important  evidence  of 
spastic  disturbance  after  lesion  of  the  pyramidal  tracts  has,  however,  been 
absent  in  some  cases  of  pure  lateral  sclerosis,  or  found  in  a  less  clearly  defined 
form.  The  appearance  of  a  powerful  reflex  dorsal  flexion  of  the  foot  and 
toes  upon  irritation  of  the  inner  surface  of  the  leg  proper  is  also  of  great 
diagnostic  importance  (Oppenheim's  dorsal  leg  phenomenon).  Occasion- 
ally also,  especially  in  younger  patients,  there  appears,  upon  irritation  of  the 
sole  of  the  foot,  a  spreading  of  the  toes  with  particularly  energetic  abduction 
of  the  small  toe  (Babinski' s  fan  phenomenon),  which  may,  moreover,  be 
combined  with  peculiar  clonic  twitchings  of  the  toes. 

In  the  ascending  progress  of  the  disease,  the  phenomena  of  hypertonia, 
paresis,  and  exaggeration  of  the  tendon  and  periosteal  reflexes  extend  also 
to  the  upper  extremities.  Then  the  arms,  too,  show  changes  in  posture,  with 
abduction  of  the  upper  arm,  flexion  of  the  forearm  and  pronation  of  the 
hand,  while  the  muscular  weakness  is  evinced  preponderantly  in  lifting 
and  extending  the  arm  and  on  dorsal  flexion  of  the  hand.  The  arm  reflexes 
are  extraordinarily  active;  hand  clonus  may  be  brought  about  by  brusque 
dorsal  flexion  of  the  hand  and  flexion  of  the  fingers  by  tapping  the  back  of  the 
hand.  The  very  rare  skin  reflexes  in  the  arms,  shown  by  abduction  and 
extension  of  the  arm,  and  spreading  of  the  extended  fingers  upon  irritation 
of  the  palm  of  the  hand,  have  been  but  little  studied. 

Finally,  the  motor  cerebral  nerves  may  also  be  attacked;  we  may  conclude 
that  they  are  spastically  disturbed  from  the  presence  of  a  strangely  tense 
expression  in  the  face,  from  difficulty  in  and  inhibition  of  speech,  from  diffi- 
culty in  the  movements  of  the  muscles  concerned  with  chewing  and  swallow- 
ing, as  well  as  from  attacks  of  spasm  of  the  glottis.     In  such  cases,  the  loss 


288  DISEASES  OF  THE  SPINAL  CORD 

of  central  inhibition  of  expressive  and  emotional  movements,  is  evinced  in 
remarkable  fashion  by  the  appearance  of  compulsory  laughing  and  crying, 
induced  by  slight  stimulation  and  sometimes  lasting  without  cessation,  as 
long  as  one  occupies  one's  self  with  the  patient.  When  the  disturbance  of  the 
entire  central  motor  system  of  the  pyramidal  tracts  is  extensive,  the  disease 
usually  shows  a  certain  tendency  to  extend  to  the  spinal  motor  centers. 
This  may  be  recognized  by  the  localized  muscular  atrophy  that  appears  in 
places,  especially  in  the  hands  (cf.  also  Fig.  64  a).  These  cases  represent 
transitions  to  amyotrophic  lateral  sclerosis.  In  pure  cases  of  primary 
lateral  sclerosis,  the  conservation  of  the  muscles  and  the  absence  of  all 
localized  atrophy  is  characteristic. 

In  all  cases  of  spastic  spinal  paralysis,  the  functions  of  superficial  and 
of  the  deep  sensibility  remain  absolutely  intact.  No  vasomotor  nor  trophic 
disturbances  are  observed,  the  organs  of  sense  suffer  no  sort  of  injury, 
disturbances  of  the  bladder  and  intestinal  tract  are  absent.  At  most,  when 
the  spasms  are  very  severe,  possibly  because  of  the  participation  in  the 
general  rigidity  of  the  muscles  of  the  floor  of  the  pelvis,  there  may  result 
difficulty  in  micturition. 

Pathological  Anatomy. — The  symptom  complex  of  spastic  spinal 
paralysis  is  produced  by  a  degeneration  of  the  pyramidal  tracts,  which,  in 
the  pure  cases,  may  be  traced  through  the  entire  spinal  cord,  in  the  lateral 
columns,  and  even  in  the  anterior  columns  (Fig.  64).  The  manner  and 
duration  of  the  disease  determine  the  spread  of  this  degeneration  to  the 
higher  sections  of  the  central  motor-conducting  tracts.  Occasionally  it 
extends  beyond  the  pyramidal  crossing  to  the  pons,  the  cerebral  peduncles 
and  the  internal  capsule.  The  ganglion  cells  of  the  psychomotor  fields  of  the 
cerebrum,  are,  as  a  rule,  found  intact,  nor  does  the  lesion  extend  to  the  motor 
ganglion  cells  in  the  spinal  centers  of  the  anterior  columns,  that  is,  their 
peripheral  branches  in  the  anterior  roots  and  the  peripheral  nerves.  Even 
the  muscles  themselves  prove  upon  anatomic  examination  to  be  intact;  in 
fact  the  muscle  fibres  are  especially  voluminous  and  hypertrophic  in  the 
regions  most  affected  by  the  hypertonia  and  the  exaggeration  of  the  reflexes. 
Transitions  to  combined  column  diseases  of  the  spinal  cord  may  be  observed, 
due  to  the  presence  of  a  slight  systemic  degeneration  in  the  posterior  columns, 
especially  affecting  the  funiculi  graciles  (Goll),  but  in  these  cases,  owing  to 
the  precedence  in  time  and  the  permanent  preponderance  of  the  functional 
disturbances  of  the  pyramidal  tracts,  the  spastic  paretic  symptom  complex 
comes  so  completely  to  the  fore,  that  the  phenomena  of  sensory  loss  of 
function,  ataxic  disturbances,  and  diminution  in 'the  tendon  reflexes  seem 
to  be  wholly  concealed. 

^Etiology. — The  familial  and  hereditary  occurrence  of  a  pure  spastic 
spinal  paralysis,  i.  e.,  primary  lateral  sclerosis,  observed  by  v.  Strilmpell  and 
others,  places  the  possibility  of  a  definitely  endogenous  occurrence  of  this 


ETIOLOGY  AND  DIAGNOSIS  OF  SPASTIC  SPINAL  PARALYSIS   289 

systemic  disease  beyond  doubt.  But  also  in  the  isolated  cases,  probably  a 
deficient  development  of  the  tract,  which  later  is  destroyed,  gives  the  founda- 
tion for  the  elective  disease,  which  is  frequently  excited  in  more  mature  age 
under  exogenous  influences,  like  intoxications,  infections,  nutritional  dis- 
turbances (anemia),  exhausting  over-exertions,  and  traumatisms  with  their 


FlG.  64. — Primary  pyramidal-lateral  column  sclerosis  in  spastic  spinal  paralysis.  Weigert- 
Pal  staining,  a,  Upper  cervical  cord.  Besides  the  degeneration  of  the  pyramidal  lateral  tracts  a 
slight  atrophy  of  the  anterior  columns  exists;  b,  lower  cervical  cord;  c,  upper  dorsal  cord;  d, 
lower  dorsal  cord;  e,  lumbar  cord.     (Erlangen  Medical  Clinic?) 

consequent  phenomena.  The  injuries  produced  as  the  after-effects  of 
syphilitic  infection,  not  infrequently  remain  for  a  long  time  confined  to  the 
pyramidal  tracts;  this  is  likewise  reported  of  chronic  lead  poisoning,  of 
eating  lathyrus  seeds  (lathyrus  sativus,  lathyrus  cicera,  chick  pea),  of 
pellagra  occurring  in  those  living  upon  Indian  corn,  of  phenomena  conse- 
quent to  the  puerperium  and  to  other  infectious  diseases. 

Differential  Diagnosis. — The  clinical  picture  of  spastic  spinal  paralysis 
is  so  characteristic  in  its  combination  of  muscular  rigidity,  reflex  exaggera- 
19 


29o  DISEASES  OF  THE  SPINAL  CORD 

tionj  and  typically  distributed  pareses  and  contractures,  that  it  can  scarcely 
fail  to  be  recognized  symptomatologically.  The  disturbances  that  take  place 
in  active  and  passive  motility,  the  increase  in  the  tendon  reflexes  and  the 
pseudoclonus  which  are  met  with  in  hysteric  and  psychopathic  patients 
(medico-legal  accident  patients)  can  not  easily  be  mistaken  for  it  if  the 
arrangement  and  localization  of  the  disturbances  in  movement  are  carefully 
studied,  the  cutaneous  reflexes  (BabinskV  s  and  Oppenheim's  signs)  carefully 
tested  and  the  associated  movements  taken  into  consideration.  In  this 
respect  serious  difliculties  in  diagnostic  judgment  arise  only  when  the 
organic  disturbances  are  concealed  or  blurred  by  a  hysteria  that  has  super- 
vened upon  the  organic  disease. 

More  difficult  and  often  answerable  only  after  long  and  thorough  observa- 
tion is  the  question,  whether  the  spastic-paretic  symptom  complex  is  pro- 
duced by  a  primary  pure  lateral  sclerosis,  or  whether  we  are  dealing  with  a 
symptom  of  some  other  cerebral  or  spinal  disease.  The  diagnosis  of  a 
primary  lateral  sclerosis  can  and  ought  to  be  made  only,  then,  if  after  careful 
consideration  of  the  subjective  disturbances,  and  after  minute  examination 
of  the  sensibility,  co-ordination,  reflexes,  the  trophic  and  vasomotor  relations, 
the  spinal  column  and  the  cerebro-spinal  fluid,  the  cerebral  nerves  and  the 
organs  of  special  sense  (the  ophthalmoscopic  findings),  the  urogenital  and 
intestinal  functions,  especially,  too,  after  complete  clearing  up  of  the  previous 
history,  especially  of  the  course  of  the  disease,  a  symptomatic  occurrence  of 
spastic  paresis  in  myelitis,  compression  myelitis,  cerebro-spinal  syphilis, 
hydrocephalus,  spinal  gliosis,  progressive  paralysis,  multiple  sclerosis, 
encephalitis  and  other  diseases,  may  be  excluded. 

In  old  age  there  develops  occasionally  a  spastic  paraparesis  with  contrac- 
tures, typical  reflex  changes  and  a  disturbance  in  walking,  which  causes  the 
gait  to  resemble  that  of  the  spastic  because  of  its  stiffness  and  weakness, 
which  is  accompanied  by  an  attenuation  of  the  pyramidal  tract  areas  in  the 
lateral  columns  and  is  probably  brought  about  by  nutritional  disturbance 
in  the  spinal  cord  induced  by  arterio-sclerotic  changes,  though  it  may  also 
have  its  seat  in  the  cerebral  cortex. 

The  prognosis  of  spastic  spinal  paralysis  naturally  depends  upon  the 
causes  of  the  symptom  complex.  In  the  cases  of  pure  lateral  sclerosis,  it  is 
not  unfavorable,  in  that  while  the  course  of  the  disease  is  progressive,  it  is 
frequently  very  protracted.  The  disease  sometimes,  too,  may  come  to  a 
standstill  for  years  and,  as  a  rule,  does  not  affect  the  vital  nervous 
functions. 

The  therapy  can,  to  a  certain  degree,  at  least,  be  causal,  inasmuch  as 
it  seeks  to  prevent,  by  rest  and  nerve  economy,  a  further  exhaustion  of  the 
weakened  motor  system.  Therefore  the  patient,  even  at  the  beginning 
of  the  disease,  must  avoid  all  movements  of  the  body,  that  stir  him  up  to 
overhaste  or  over-exertion,  or  which  by  the  tendency  to  make  him  fall,  might 


TREATMENT  OF  SPASTIC  SPINAL  PARALYSIS  291 

cause  traumatic  injuries.  He  must  also  avoid  long  walks  and  mechanical 
occupations  requiring  the  frequent  repeated  use  of  certain  muscles.  On  the 
other  hand,  by  careful  practice  in  walking  and  standing,  at  first,  with  the 
assistance  of  passive  movement  by  stimulating  the  cutaneous  reflexes,  which 
often  materially  aid  the  flexing  movements  of  the  legs,  the  faculty  for  active 
movement  can  be  improved  methodically  and  by  becoming  accustomed  to 
the  altered  circumstances,  some  degree  of  freedom  in  movement  may  be 
attained.  It  is  desirable  as  well  that  the  patient  should  become  aware  of  the 
possibility  of  substitution  movements,  which  may  almost  always  be  used,  and 
perfect  these  gradually  by  use;  for  this,  however,  if  he  remains  a  long  time 
in  bed,  he  will  lose  both  the  opportunity  and  energy.  Since,  with  the  weak- 
ening of  the  cutaneous  irritability  and  the  lessening  of  the  effect  of  weight 
in  the  warm  bath,  the  stiffness  in  the  muscles  and  the  spasticity  gradually 
give  way,  the  persistent  use  of  protracted  warm  baths  is  to  be  recommended. 
At  the  same  time,  the  patients  may  derive  benefit  from  the  vivifying  influence 
of  carbonic  acid  baths  at  home,  or  at  suitable  health  resorts.  Careful 
massage,  and  especially  passive  aid  in  attempts  at  active  movement,  even 
in  the  bath  itself,  may  further  the  motility,  at  least  in  the  first  stages  of  the 
disease.  With  the  lowering  of  the  tonic  excitability  by  medicines  (morphine, 
narcotizing  injections  into  the  dural  sac)  one  will  have  to  be  most  conserva- 
tive, considering  the  duration  of  the  disease.  It  is  far  more  important  to 
develop  by  suitable  means  the  active  powers  of  the  patient  in  overcoming 
muscular  resistance.  For  this  purpose  one  may  use  moderate  application 
of  the  constant  current,  while  the  stimulation  of  faradization  and  great 
changes  in  current  are  to  be  avoided.  Spastic-paretic  patients  doubtless 
often  recover  their  ability  to  move  to  a  signal  extent,  if  they  leave  externally 
unfavorable  environments  and  go  to  a  place  where  they  can  have  good 
physical  care  and  better  nourishment. 

The  results  of  orthopaedic  surgery  have  not  been,  so  far,  very  encourag- 
ing in  spastic  pareses,  and  especially  in  spastic  spinal  paralysis,  unless  we 
are  concerned  about  getting  rid  of  troublesome  phenomena  of  secondary 
importance,  as  contractures.  Neither  through  apparatus  for  support  and 
posture,  nor  by  cutting  tendons  and  muscles,  nor  by  plastic  and  grafting 
operations,  can  one  effectively  combat  the  disproportion  between  the 
increased  reflex  muscle  tonus  in  some  and  the  diminution  of  the  voluntary 
development  of  strength  in  other  groups  of  muscles.  Recently,  it  has  been 
attempted  successfully,  after  laminectomy,  to  resect  part  of  the  posterior 
roots  in  corresponding  selection,  and  thereby,  without  causing  phenomena 
of  serious  functional  loss,  to  weaken  the  reflex  exaggerations  and  the  hyper- 
tonia to  such  an  extent,  that  the  active  capacity  for  movement  becomes 
more  free  (Forster).  In  desperate  cases,  this  method  must  be  considered 
as  the  ultima  ratio,  if  there  be  a  possibility  of  strict  aseptic  procedure  and 
most  careful  after-treatment. 


292  DISEASES  OF  THE  SPINAL  CORD 

(d)  Amyotrophic  Lateral  Sclerosis 

BY 

FR.  JAMIN  (Erlangen) 

The  relatively  rare  disease,  amyotrophic  lateral  sclerosis,  affecting  men 
more  frequently  than  women,  is  a  comparatively  rapidly  advancing  destruc- 
tion of  almost  the  entire  motor  central  nervous  system,  occurring  in  mature 
age — between  the  thirtieth  and  fiftieth  years.  In  this  affection  not  only  the 
cortico-spinal  motor  conduction  path  perishes,  especially  in  its  most  impor- 
tant component,  the  pyramidal  tracts,  but  also  the  spinal  motor  centers  in 
the  gray  anterior  horns  of  the  spinal  cord,  and  the  nuclei  of  the  motor  cerebral 
nerves  situated  in  the  medulla  oblongata.  In  the  clinical  picture  the  injury 
of  the  first  central  motor  neuron  causes  the  spastic  paretic  symptom  complex, 
as  is  shown  in  a  pure  form  in  spastic  spinal  paralysis,  with  muscular  rigidity, 
exaggeration  of  reflexes  and  diminution  in  ability  to  perform  voluntary 
movements — while  the  loss  of  the  second  peripheral  neuron  is  responsible 
for  the  symptom  complex  of  paralysis  and  wasting  of  the  muscles  with  the 
reaction  of  degeneration. 

One  would  think  that  the  latter  should  bring  about  a  disappearance  of 
the  spastic  phenomena.  But  since  to  the  extensive  injury  of  the  reflex 
inhibiting  cortico-fugal  motor  paths,  even  in  the  advanced  cases,  there  is 
opposed  only  a  partial  injury  of  the  spinal  and  bulbar  nuclei,  which  is 
confined  to  the  innervation  sphere  and  the  trophic  influence  of  special 
groups  of  muscles,  even  parts  of  muscles,  there  exists  in  amyotrophic  lateral 
sclerosis  an  intimate  union  of  spastic  symptoms  with  paralysis  and  so-called 
"degenerative"  muscular  atrophy,  which  is  the  distinctive  characteristic  of 
this  disease.  Nor  does  the  lesion  affect  all  parts  of  the  motor  system  in  the 
same  way  from  the  beginning,  but,  on  the  contrary,  according  to  the  localiza- 
tion of  the  injury,  definite  types  may  be  distinguished,  which,  in  the  majority 
of  cases,  denote  successive  stages  of  the  disease: 

i.  The  paralysis  and  muscular  atrophy  in  the  arms  combined  with 
spastic  disturbances. 

2.  The  disturbances  in  the  movements  of  the  legs,  corresponding  more 
particularly  to  the  spastic  symptom  complex. 

3.  The  atrophic  paralysis  in  the  area  of  the  motor  cerebral  nerves  appear- 
ing also  with  symptoms  of  spasms,  bulbar  paralysis. 

Some  observations  of  its  occurrence  as  a  familial  affection,  permit  us  to 
conclude  that  the  cause  of  this  exhaustion  associated  with  degeneration  of 
the  motor  nervous  system  at  the  time  of  active  life,  may  rest  upon  a  con- 
genital weakness  of  this  system.  Physical  over-exertions  and  the  influence 
(interpreted  with  difficulty)  of  colds  and  traumatisms  are  stated  as  factors 
in  exciting  the  disease.  It  is  not  known  whether  a  specific  uniform  cause  of 
the  disease  is  predispositional  to  its  development. 


AMYOTROPHIC  LATERAL  SCLEROSIS 


2  93 


Symptomatology  and  Course  of  the  Disease. — The  insidious  develop- 
ment of  the  disease  makes  itself  in  most  instances  first  known  by  a  functional 
disturbance  of  the  upper  extremities.  In  occasional  cases,  however,  the 
disease,  even  at  the  beginning  shows  a  unilateral  type,  and  attacks  the  arm 
and  leg  on  one  side  at  approximately  the  same  time.  Even  when  it  begins 
in  the  arms  the  disturbance  on  the  one  side  (mostly  the  right)  as  a  rule 
precedes  that  on  the  other  by  some  months. 

The  patients   themselves  notice,   that  their  arms   and  hands  become 
weaker  and  less  skilful  at  their  usual  work,  that  they  are  easily  fatigued, 
without  the  occurrence,  however,  of  pains  or  paresthesias.     In  the  further 
course,  after  some  months,  or  only  after  a 
space  of  a  year,  there  is  noticeable  a  disturb- 
ance in  gait,  an  unusual  fatigue  after  walk- 
ing, with  stiffness,  clumsiness,  and  weakness 
in  the  legs. 

The  objective  findings  exhibit  early  in  the 
arms  increased  muscular  tension  with  a  ten- 
dency to  active  contractures,  especially  with 
regard  to  an  adduction  of  the  upper  arm, 
flexion  of  the  forearm,  pronation  of  the  hand 
and  flexion  of  the  phalanges.  Synchronously 
the  characteristic  muscular  atrophy  is  found, 
at  first,  in  the  small  muscles  of  the  hand. 
The  balls  of  the  thumbs  and  of  the  small 
fingers  become  flatter.  The  wasting  of  the 
interossei  gives  rise  to  significant  deep  hollows 
on  the  back  of  the  hand,  the  adductor  pollicis 
becomes  thin  (cf.  Fig.  65).  Next,  the  ex- 
tensors   in    the    forearm    become    atrophic 

followed  by  the  long  muscles  of  the  thumb,  then,  too,  the  rest  of  the  mus- 
cles of  the  radial  region  and,  since  the  flexors  of  the  hand  and  the  fingers 
are  relatively  better  preserved  and  remain  more  effective,  there  results  a 
claw-like  position  or  flexion  contracture  of  the  fingers.  Afterwards,  the 
deltoid  and  the  triceps  become  visibly  atrophic,  finally,  the  rest  of  the  muscles 
of  the  arms  and  the  shoulder  girdle.  Associated  with  the  muscular  wasting, 
there  is  a  muscular  weakness  which  ends  in  complete  paralysis,  and  even  the 
muscles  which  have  retained  their  volume  relatively  well,  frequently  evince  a 
considerable  diminution  in  power. 

Upon  careful  observation  of  the  diseased  muscular  parts  one  sees, 
especially  plainly  in  connection  with  attempts  at  movement,  a  lively  play  of 
fibrillary  and  fascicular  twitchings.  The  electric  excitability  is  lowered,  but  for 
the  most  part  only  in  quantitative  proportion  to  the  wasting  of  the  muscle 
fibres.     In  the  more  severely  atrophied  muscles,  however,   especially  in 


Fig.  65. — Hand  in  amyotrophic 
lateral  sclerosis.  Flexing  contracture 
of  the  end  phalanges,  II-V.  At  the 
place  of  the  adductor  pollicis  and  the 
interossei  visible  hollows.  ( Erlangen 
Medical  Clinic.) 


2Q4  DISEASES  OF  THE  SPINAL  CORD 

those  of  the  hand,  a  total  or  at  times  only  a  partial  reaction  of  degeneration 
may  be  observed. 

The  tendon  and  periosteal  reflexes  are  always  very  markedly  exaggerated 
in  the  weakened  and  atrophied  arms.  From  the  radius  and  the  ulna,  and 
from  the  tendons  of  the  upper  arm  muscles,  especially  in  the  triceps,  lively 
reflex  muscular  twitchings  can  be  evoked;  even  wrist  clonus  is  observed. 

Sensibility  remains  absolutely  undisturbed  in  all  its  qualities. 

While  in  the  arms,  the  muscular  wasting  and  the  paralysis  preponderate 
early  over  the  spastic  phenomena,  without  wholly  crowding  them  out,  the 
lower  extremities,  should  they  share  in  the  further  course  of  the  affection, 
exhibit  the  reverse.  Here,  the  spastic-par etic  phenomena  step  quite  decidedly 
to  the  fore,  whereas  the  muscular  atrophy,  fibrillary  twitchings  and  changes  in 
electrical  excitability  appear  either  not  at  all,  or  relatively  late,  and  then 
mostly  in  the  muscles  of  the  leg  proper.  The  laborious,  dragging,  sliding 
gait,  the  paresis  of  the  flexors,  group  action  of  the  extensors  and  flexors  of  the 
limb,  and  associated  movements  (tibial  and  toe  phenomenon)  spontaneous 
clonus  and  marked  increase  of  all  tendon  reflexes,  changes  in  the  cutaneous 
reflexes  (Babinski's  great  toe  reflex  and  Oppenheim's  leg  reflex),  finally,  too, 
contractures  unite  with  the  general  hypertonia  of  the  muscles  of  the  leg, 
which  is  made  still  greater  by  skin  stimuli  and  motion,  to  make  the  clinical 
picture  of  spastic  paraparesis.  In  the  trunk,  and  the  legs,  also,  disturbances 
of  sensation  are  altogether  absent.  With  the  exception  of  a  slight  difficulty 
in  urination  and  defecation  (constipation)  due  to  spasms  in  the  musculature 
of  the  pelvic  floor,  there  appear  no  bladder  or  intestinal  disturbances. 

After  the  patient,  by  a  combination  of  spastic  paraparesis  and  a  more  or 
less  complete  inability  to  use  the  upper  extremities,  has  already  reached  a 
very  helpless  condition  and  has  become  bedridden,  the  disease  becomes 
really  tormenting  and  even  dangerous,  when,  after  one  or  two  years,  the  last 
group  of  symptoms,  which,  in  many  cases,  however,  has  developed  much  earlier, 
namely,  that  of  bulbar  paralysis,  is  added  to  the  other  symptoms. 

Now,  with  the  paralysis  that  appears  in  the  area  of  the  motor  cerebral 
nerves,  and  the  muscular  atrophy,  speech  disturbances  develop;  speech 
becomes  first  nasal  and  difficult  to  understand,  is  articulated  worse  and 
worse,  finally  becomes  aphonic;  there  appear  difficulty  in  swallowing,  paralysis 
of  the  soft  palate,  of  the  tongue  with  visible  atrophy  and  fibrillary  twitchings,  of 
the  muscles  of  chewing,  and  of  the  facial  muscles,  especially  in  the  lower  half 
of  the  face.  The  lips  become  thin  and  can  no  longer  be  raised  and  pursed  at 
will,  whereas  the  expressive  movements  of  the  face  are  frequently  retained. 
In  these  regions  also  side  by  side  with  and  often  before  the  appearance  of 
atrophy  and  paralysis,  the  loss  of  cerebral  inhibition  makes  itself  felt  in  many 
ways:  the  masseter  reflexes  are  exaggerated;  the  muscles  of  the  face  are  tense, 
often  spasmodically  distorted;  glottis  spasms  appear,  sudden  and  apparently 
causeless  bursts  of  emotion  (obsessional  laughing  and  crying)  with  retention 


AMYOTROPHIC  LATERAL  SCLEROSIS  295 

of  the  intellect  indicate  the  increase  in  reflex  excitability.  In  the  wrinkled 
tongue,  which  is  moved  with  difficulty  and  in  the  lips  and  the  muscles  of  the 
pharynx  the  reaction  of  degeneration  or  at  least  slow  contraction  upon  galvanic 
stimulation  may  be  demonstrated. 

Only  the  muscles  of  the  eye  are,  for  the  most  part,  spared.  The  mechan- 
ism of  respiration  may  be  impaired  by  the  co-affection  of  the  diaphragm  and 
the  muscles  of  the  ribs,  so  that  the  patient  finally  succumbs  to  a  respiratory 
paralysis,  unless  the  secondary  phenomena  of  paralysis  of  the  throat  and  the 
swallowing  apparatus  (aspiration  pneumonia)  and  the  disturbances  in  nour- 
ishment, necessarily  connected  with  the  bulbar  disturbances,  have  already 
caused  death  after  a  few  years. 

The  course  of  the  disease  and  the  order  of  the  appearance  of  the  individual 
symptoms  differ  in  different  cases,  depending  upon  whether  the  lesion  of  the 
pyramidal  tract  or  the  loss  of  the  motor  nuclei  in  the  medulla  oblongata  and 
in  the  spinal  cord  has  temporal  precedence,  and  according  as  to  which  retains 
the  preponderant  importance  for  a  time,  or  permanently.  Thus,  there  are 
found  transitional  forms  to  spastic  spinal  paralysis,  in  which  the  atrophic 
paralysis  remains  altogether  undeveloped,  to  progressive  muscular  atrophy, 
in  which,  from  the  beginning,  the  paralysis  with  muscular  wasting  conceals 
the  spasticity,  or  stops  it  in  the  final  stage  of  the  disease,  and  to  progressive 
bulbar  paralysis,  in  which,  early,  the  paralysis  of  the  cerebral  nerves, 
accompanied  by  slight  spastic  disturbances  in  the  extremities,  comes  to  the 
foreground. 

Pathological  Anatomy. — Transverse  section  of  the  spinal  cord  (Fig. 
66)  shows  in  amyotrophic  lateral  sclerosis  very  distinctly,  especially  in  the 
cervical  segment,  the  combination  of  atrophy  of  the  pyramidal  tracts  with 
atrophy  of  the  anterior  horns,  corresponding  to  the  spastic  atrophic  paralysis 
which  is  most  pronounced  in  the  arms.  The  pyramidal  tracts  have  wasted 
in  the  lateral  column  and,  occasionally,  also  in  the  anterior  column,  not 
here  only  but  in  their  entire  spinal  course,  and  in  their  ascending  way 
through  the  medulla  oblongata,  the  pons  up  to  the  cerebral  peduncle  and 
the  inner  capsule.  Slight  wasting  of  fibres,  too,  is  noticeable  in  the  rest  of 
the  area  of  the  antero-lateral  column,  in  some  cases,  also  in  the  medial 
posterior  column,  without  the  appearance,  however,  of  noticeable  sensory 
phenomena  of  functional  loss.  The  gray  matter  of  the  anterior  columns 
becomes  reduced,  especially  in  the  cervical  segment,  by  the  wasting  of  its 
network  of  nerve-fibres  with  the  exception  of  the  reflex  collaterals  (Oppen- 
heim)  and  by  destruction  of  a  large  part  of  the  large  motor  ganglion  cells. 
The  anterior  roots,  too,  usually  become  smaller.  A  corresponding  nuclear 
wasting  may  be  observed  also  in  the  bulbar  centers  of  several  cerebral  nerves, 
in  the  nucleus  of  the  hypoglossus,  the  vagus-accessorius,  the  motor  part  of  the 
trigeminus,  and  in  a  slighter  degree  also  in  the  facial.  The  psychomotor 
cortical  fields  and  their  nearest  connection  paths,  also,  have  not  always  been 


296  DISEASES  OF  THE  SPIXAL  CORD} 

found  intact.     Slight  proliferation  of  the  supporting  connective  tissue   re- 
places the  nervous  elements  that  are  lost. 

To  the  extensive  loss  of  the  spinal  motor  centers,  correspond  degenera- 
tions in  the  peripheral  motor  nerves,  which,  however,  are  difficult  to  find  in 
the  mixed  nerve  trunks,  and  also  because  of  the  mingling  of  fibres  still 
capable  of  conduction  with  those  that  are  already  incapable.  In  the  atro- 
phied muscles  of  the  arm,  the  •wasting  of  muscular  fibres,  resulting  in  some 
places  in  complete  loss  of  the  contractile  substance  with  increase  of  the 
muscle  nuclei  and  the  interstitial  connective  tissue,  mav  be  demonstrated 


FiG.  66. — Amyotrophic  lateral  sclerosis.  Weigert  staining.  Uppermost  cervical  cord.  The 
pyramidal  tracts  show  degeneration  in  the  anterior  and  lateral  columns.  A  slight  degeneration  is 
to  be  found  also  in  Goll's  column.  The  gray  anterior  columns  have  almost  disappeared.  {After 
Schmaus.) 

easily,  accompanied  under  certain  circumstances  by  profuse  infiltration 
of  fat.  In  some  muscles,  affected  only  by  partial  atrophy,  more  or 
less  atrophied  muscular  fibres  are  mingled  with  those  that  are  well 
preserved. 

In  the  differential  diagnosis  of  amyotrophic  lateral  sclerosis  it  is 
especially  important  to  note  that  this  disease  is  strictly  confined  to  motor 
functional  disturbances.  Testing  the  sensation  and  consideration  of  the 
subjective  troubles,  which  are  not  to  be  explained  by  disturbances  in  move- 
ment, like  pains  and  paresthesias,  make  easily  possible  a  distinction  from 
the  other  spinal  troubles,  which  like  syringomyelia,  spinal  tumors,  cervioal 
pachymeningitis,  myelitis  localized  in  the  cervical  segment,  or  syphilis  dna 
tuberculosis  of  the  cord  may  be  accompanied  by  similar  atrophic  paralyses 


SPINAL  PROGRESSIVE  MUSCULAR  ATROPHY  297 

in  the  arms  with  spasticity  in  the  legs.  Differentiation  from  the  rest  of  the 
system  motor  diseases  is  made  possible  by  the  subacute  course  and  the 
commingling  of  spastic  phenomena  with  cervical  and  bulbar  atrophic  paralyses. 
In  regard  to  this  the  increase  of  the  periosteal  reflexes  in  the  atrophied  fore- 
arms is  especially  significant  for  the  myatrophy  connected  with  lateral 
sclerosis;  it  does  not  appear  in  like  manner  in  spinal  muscular  atrophy,  nor 
in  other  lesions  restricted  to  the  anterior  horns,  due  to  spinal  gliosis  or 
hgematomyelia.  In  multiple  sclerosis,  which  not  infrequently  simulates 
motor  systemic  diseases,  localized  muscular  atrophies  are  scarcely  to  be 
observed  in  similar  arrangement  and  development;  it  may  be  recognized 
frequently,  besides,  by  proof  of  disturbances  in  co-ordination  and  due 
consideration  of  the  eye  findings  even  in  the  atypical  cases. 

The  prognosis  of  amyotrophic  lateral  sclerosis  is  in  every  respect  un- 
favorable. Irresistibly,  and  apparently  indifferent  to  external  influences, 
the  disease  proceeds  to  its  fatal  end. 

The  treatment  can  therefore  only  be  symptomatic.  Consideration  and 
good  care  are  obviously  necessary  because  of  the  helpless  condition  of  the 
patient.  In  the  final  stages,  the  patients,  under  certain  conditions,  have  to 
be  fed  through  a  tube.  The  spasticity  and  contractures  may  be  combated 
by  baths,  careful  massage,  also  by  suitable  bandages;  because  of  the  progres- 
sive nature  of  the  disturbances,  it  is  better  to  abstain  from  operative  treat- 
ment. The  employment  of  the  constant  current  in  the  region  of  the  nape 
and  along  the  spinal  column,  also  in  the  area  of  the  atrophied  muscles,  and 
the  administering  of  nourishing  foods  and  strengthening  medicines,  etc.,  is 
to  be  all  the  more  recommended  since  only  steadily  continued  therapeutic 
procedures  may  spare  the  patient,  who  with  unimpaired  intellect  is  gradually 
perishing,  a  clear  insight  into  his  condition. 

(e)  Spinal  Progressive  Muscular  Atrophy  (Duchenne-Aran) 

BY 

FR.  JAMIN  (Erlangen) 

The  spinal  motor  centers  in  the  gray  anterior  horns  of  the  spinal  cord 
with  their  thick  network  of  nerve  fibres  and  the  groups  of  motor  ganglion 
cells  are  the  last  central  terminal  stations  for  the  muscles,  through  which  the 
stimulations  emerging  from  the  superior  centers  of  the  brain,  the  cerebellum 
and  the  subcortical  ganglia,  as  well  as  the  reflex  impulses  created  in  the 
spinal  cord  itself  must  take  their  way.  Their  destruction  causes  a  secondary 
degeneration  of  the  peripheral  motor  nerve  fibres  and  a  complete  exclusion 
of  the  attendant  contractile  muscular  fibres  from  every  voluntary  and  reflex 
activity,  that  is,  not  only  a  cessation  of  the  motor  activity,  which  has  visible 
results,  but  the  loss  of  every  function,  and  therefore  also  of  the  tension  that 


298  DISEASES  OF  THE  SPINAL  CORD 

exists  in  times  of  rest.  The  consequence  of  this  loss  of  all  functional  stimula- 
tions, is,  for  the  muscle,  a  considerable  diminution  of  the  processes  necessary 
to  its  existence,  of  circulation  and  nourishment.  Since  such  a  muscle  now 
lacks  the  power  of  an  up-building  restitution  to  the  natural  loss  of  its  sub- 
stance, a  power  afforded  it  by  its  normal  specific  function,  it  perishes  after 
the  loss  of  its  innervating  and  thereby  trophically  acting  spinal  center,  in  a 
slow  way  and,  if  considered  for  short  periods  of  time,  scarcely  perceptibly, 
yet  irresistibly,  until  the  complete  process  of  destruction,  atrophy,  is 
reached. 

An  injury  of  the  anterior  columns  of  the  spinal  cord  with  the  corresponding 
secondary  phenomena  in  the  muscles — paralysis,  loss  of  reflex  excitability  and 
muscular  wasting — occurs  in  numerous  diseases  of  the  spinal  cord.  Even 
among  the  system  motor  diseases  of  the  spinal  cord  it  has  already  been 
mentioned  as  a  partial  phenomenon  of  amyotrophic  lateral  sclerosis.  Pure 
primary  degeneration  of  the  spinal  motor  centers,  the  premature  using-up  and 
gradually  progressing  destruction  of  these  important  motor  central  stations 
is,  however,  observed  in  the  very  rare  disease  picture  of  spinal  progressive 
muscular  atrophy. 

An  entirely  sharp  separation  of  the  manifold  forms,  under  which  a  con- 
genital weakness  of  the  nervous  and  muscular  apparatus  of  motion  appears 
clinically,  can  certainly  not  be  made.  As  we  have  seen  that  the  injury  of 
the  cortico-fugal  motor  tracts  often  does  not  halt  when  reaching  the  spinal 
and  bulbar  nuclei,  but  changes  these  also  functionally  and  morphologically, 
just  so  the  atrophic  processes  of  the  myopathic  forms  of  progressive  muscular 
atrophies,  which  begin  in  the  muscles,  lead  not  infrequently  to  an  impair- 
ment of  the  function  and  the  anatomic  condition  of  the  gray  anterior  horns. 
Therefore,  even  in  this  respect,  in  a  large  number  of  transitional  forms, 
which  frequently  make  the  interpretation  of  an  individual  case  more  difficult, 
the  theory  of  the  unity  of  the  entire  motor  system,  possibly  also  a  unity  of  the 
pathological  processes  holds  good.  Notwithstanding  this,  a  considerable 
number  of  observations  during  a  protracted  course  of  the  disease  and  with 
characteristic  grouping  of  symptoms,  the  peculiar  localization  of  the  muscu- 
lar atrophy,  made  sure  by  anatomic  findings,  justifies  the  demarcation  of 
progressive  spinal  muscular  atrophy  as  a  different  group  of  diseases  from  the 
related  affections,  amyotrophic  lateral  sclerosis  and  myopathic  muscular 
atrophy. 

There  is  but  little  known  about  the  causes  of  the  disease.  Its  appearance 
in  several  members  of  one  family,  that  is,  upon  a  common  congenital  predis- 
position, has  been  observed.  The  disease  occurs,  however,  more  frequently 
isolated,  in  adults,  and  more  frequently  also  in  men  than  in  women.  The 
exciting  influence  of  local  or  general  traumatism  is  certainly  of  importance, 
but  very  difficult  to  judge  because  of  the  unusually  slow  and  insidious  develop- 
ment of  the  disease.     This  is  likewise  true  of  the  influence  of  colds  and  in- 


SPINAL  PROGRESSIVE  MUSCULAR  ATROPHY 


299 


fectious  diseases.  It  seems  that  the  disease  may  develop  upon  the  foundation 
of  a  syphilitic  infection,  just  as  in  tabes  the  injury  of  the  nervous  system  not 
infrequently  extends  itself  to  the  spinal  motor  nuclei.  Severe  physical  work 
and  over-exertion  certainly  further  the  development  of  the  disease,  but  one 
must  beware  of  a  confusion  with  the  muscular  atrophies  in  occupational 
neuroses  {Oppenheim)  which  are  more  favorable,  prognostically  and  much 
less  severe.  Finally,  the  development  of  a  progressive  spinal  muscular  atro- 
phy in  more  mature  years,  has  been  likewise  observed  being  grafted 
on  a  defect  which  developed  in  childhood  as  the  result  of  an  anterior 
poliomyelitis. 

Symptomatology  and  Course. — It  is  especially  characteristic  of  pro- 
gressive spinal  muscular  atrophy,  that  it  does  not  at  one  fell  swoop  or  with 
giant  strides  seize  upon  large  sections  of  the  spinal  motor  centres,  but  begin- 
ning very  gradually  in  a  restricted  area, 
proceeds  to  the  disjunction  of  one  cen- 
tral element  after  another,  and  there- 
fore gives  time  for  the  development  of 
the  trinity  resulting  from  wasting  of  the 
ganglion  cells,  of  the  motor  nerve  fibres 
and  of  the  muscular  fibres,  before  new 
and  further  areas  are  attacked.  There- 
fore we  see,  that  in  most  instances  the 
phenomena  of  paralysis  and  muscular 
atrophy  appear  simultaneously,  and  that 
years  often  pass  before  a  larger  part  FlG.  67._Effeminate  hand  in  spinal  pro- 
of  the  muscles  of  the  body  participates    gressive  muscular  atrophy.     Atrophy  of  the 

ball  of  the  thumb,  of  the  little  finger,  and  of 
in  these  disturbances.  the  lumbricales.     (Erlangen  Medical  Clinic.) 

Nearly  always  the  symptoms  of 
functional  loss  appear  in  the  region  of  the  small  muscles  of  the  hands. 
The  patients  themselves  probably  notice  that  they  no  longer  possess  their 
former  skill  in  finer  movements,  and  that  they  can  no  longer  develop 
sufficient  strength  in  grasping,  reaching  for,  and  holding  objects.  Upon 
examination  at  this  time,  one  finds  even  then  an  emaciation  and  flat- 
tening of  the  ball  of  the  thumb  and  of  the  little  finger,  often  more  markedly 
developed  on  the  right  than  on  the  left  hand.  Especially  the  muscles 
of  the  thumb,  first,  the  opponens  and  abductor  brevis,  then  also  the  flexor 
brevis  and  the  adductor,  lose  their  functional  ability  and  become  thin, 
limp,  and  weak  Early,  the  long  muscles  of  the  thumb  in  the  forearm,  the 
extensor  and  abductor  pollicis  longus  are  added,  so  that  then  the  thumb 
begins  to  lie  almost  immovable  on  a  plane  with  the  metacarpus  {ape's  hand) 
(Fig.  67).  But  the  other  small  muscles  of  the  hand  are  not  spared:  the  atro- 
phy of  the  interossei  and  the  lumbricales  causes  a. prominence  of  the  lines 
of  the  bones  on  the  back  of  the  hand  and  in  the  palm,  and  together  with  the 


300  DISEASES  OF  THE  SPINAL  CORD 

paralysis  of  the  extensors  of  the  terminal  phalanges  and  the  contracture  of 
the  still  more  effective  long  flexors  and  extensors  in  the  forearm,  leads  to 
the  form  known  as  claw  hand  (main  en  griff e).  In  the  period  following, 
the  extensors  of  the  forearm  are  affected,  also  the  flexors,  the  atrophy  ap- 
pearing preferably  in  the  ulnar  division  of  the  flexors,  so  that  here,  too,  the 
contours  of  the  bones  become  more  and  more  prominent.  At  the  same 
time,  there  often  exists  and  persists  a  peculiar  diminution  of  the  extremity' 
towards  the  distal  end,  which  is,  moreover,  favored  by  the  wasting  of  the 
cushion  of  fat,  which  is  mostly  connected  with  the  atrophy  of  the  muscles 
(bird  arms).  Associated  with  the  wasting  of  the  muscles  which  continues 
for  years,  and  the  greater  and  greater  immobility  of  the  hands,  the  bones, 
too,  become  thinner,  and  appear  in  a  skiagraph  poor  in  calcium  bone- 
substance,  somewhat  wasted,  with  extremely  delicate  structural  design, 
without  usually  changing  their  general  essential  configuration. 

The  further  spread  of  the  atrophy  and  the  paralysis  does  not  ascend 
the  arm  gradually,  but  usually  by  leaps,  as  a  rule,  jumping  next  to  the  deltoid. 
With  the  emaciation,  at  first  partial,  then  total,  the  patients  lose  more  and 
more  the  ability  to  raise  their  arms  (Fig.  68) .  The  arm  hangs  down  from  the 
shoulder,  adducted,  the  use  of  the  hands  is  made  possible  with  difficulty  only 
by  the  assistance  of  the  muscles  of  the  upper  arm — which  may  for  a  long 
time  continue  to  act — and  by  swinging  motions  or  by  the  combined  employ- 
ment of  the  forearms  pressed  against  each  other.  Furthermore,  the  atrophy 
spreads  into  the  region  of  the  muscles  of  the  shoulder,  early  in  the  supra- 
and  infra-spinati,  so  that  the  contours  of  the  bony  shoulder  girdle,  especially 
of -the  scapula,  become  more  and  more  prominent;  then,  too,  in  the  cucullaris, 
whereby  the  raising  of  the  arm  and  the  turning  of  the  shoulder  are  made 
still  more  difficult;  in  the  rhomboidei,  the  latissimus  dor  si,  the  pectorales, 
and  the  muscles  of  the  back  of  the  neck,  so  that  the  head  sinks  forward  and 
can  not  be  voluntarily  raised.  If  the  atrophy  of  the  muscles  is  very  far 
advanced,  as  a  result  of  the  functional  loss  of  the  muscular  tonus,  the 
ligaments  of  the  joints  relax,  and  the  joints  become  loose.  Finally,  a 
participation  of  the  muscles  of  the  trunk  may  make  the  upright  position 
difficult,  and  lead  to  a  compensating  lordosis. 

The  lower  extremities  are  but  rarely  affected  by  the  paralysis  and  the 
muscular  wasting;  but  when  the  affection  is  of  long  standing,  there  appears, 
here  too,  and  particularly  in  those  muscles  of  the  leg  that  raise  the  foot,  and 
in  the  pelvic  muscles,  a,  flaccid  atrophic  paralysis.  Therefore  the  gait 
becomes  a  waddle,  due  to  the  loss  of  fixation  of  the  pelvis  to  the  thigh.  In 
order  to  lift  the  foot,  which  is  hanging  limply  down,  from  the  ground,  the 
thigh  must  be  raised  abnormally  high.  The  patients  become  fatigued 
easily  and  become  more  and  more  helpless.  Even  in  spite  of  the  ad- 
vanced degree  of  atrophy,  the  patients  can  help  themselves  to  a  certain 
point  and  retain  some  mobility  through  the  skillful  use  of  the  muscles 


SPINAL  PROGRESSIVE  MUSCULAR  ATROPHY 


3oi 


that  are  retained  and  still  act;  finally,  however,  such  a  large  part  of  the 
musculature  of  the  body  is  affected  that  the  patients  become  bedridden, 
and  depend  upon  the  help  of  others  for  all  that  has  to  be  done.  Participation 
in  the  disease  process  of  the  muscles  of  respiration  may  finally  lead  to  serious 
difficulty  in  breathing,  and  thereby  to  a  fatal  end.     The  condition  becomes 


Fig.  68. — Spinal  progressive  muscular  atrophy.  Besides  the  little  muscles  of  the  hand 
the  muscles  of  the  forearms  and  the  upper  arms  have  also  atrophied.  (After  Schoenborn  and 
Krieger.) 


equally  dangerous  when  the  muscular  atrophy  reaches  the  realm  of  the 
motor  cerebral  nerves  and  leads  to  the  development  of  progressive  bulbar 
paralysis  (cf.  p.  318). 

All  these  stages  of  the  disease  develop  in  the  course  of  years.  The 
reacquisition  of  the  capacity  for  movement,  when  it  has  once  been  lost,  is 
impossible;  an  improvement  is  possible  only  in  so  far  as  the  patients  may  learn 


302  DISEASES  OF  THE  SPINAL  CORD 

by  practice  and  habit,  in  adaptation  to  the  changed  circumstances,  to  put 
the  muscular  parts  that  have  been  retained  to  better  use.  Quite  often  also, 
the  condition  remains  apparently  stationary  for  a  long  time.  But  if,  at 
intervals  of  some  time,  an  exact  and  detailed  examination  is  made,  one  will 
usually  be  able  to  note  an  advance  of  the  disease. 

The  onset  of  the  disease  in  the  arms,  i.  e.,  in  the  small  muscles  of  the  hand, 
and  next  to  them  in  the  deltoid,  is  of  by  far  the  most  frequent  occurrence. 
But  cases  are  observed  also,  in  which  the  first  signs  appear  in  the  shoulder 
girdle  or  even  in  the  legs,  and  they  are  then  much  more  difficult  to  find,  than 
the  characteristic  change  in  the  configuration  of  the  hands,  which  immediately 
attracts  the  notice  of  the  skilled  practitioner.  Possibly  in  this  selective 
action,  so  far  as  localization  of  the  disturbance  is  concerned,  the  kind  of 
occupation  may  play  a  certain  role,  so  that  the  regions  most  employed  fall 
first  and  most  completely  under  the  sway  of  the  atrophic  process. 

The  paralysis  remains,  as  a  rule,  confined  to  the  atrophied  muscles,  and, 
in  its  degree,  is  dependent  upon  the  degree  of  muscular  wasting  which 
frequently  spares  isolated  bundles  of  fibres  in  the  individual  muscles,  as 
can  occasionally  be  seen  clearly  in  the  contraction  of  the  deltoid. 

In  addition  to  the  muscular  wasting,  which  is  usually  confined  within 
strict  boundaries  and  in  typical  localization,  and  the  parallel  functional 
disturbance  of  paresis,  or  paralysis  in  certain  parts  of  the  limbs,  some  further 
changes  are  worthy  of  notice. 

The  tendon  and  periosteal  reflexes  are  absent  in  the  atrophied  regions. 
In  the  arms  they  are  nearly  always  lost  or  diminished  to  a  high  degree,  and 
this  is  the  case  even  when  the  musculature  in  a  part  of  the  fore-  and  upper 
arm  is  relatively  well  retained  and  is  functionally  intact.  In  the  lower 
extremities  they  are  usually  retained  for  a  correspondingly  longer  time. 
The  cutaneous  reflexes  are  changed  only  in  so  far  as  muscles  that  have 
become  wholly  atrophied,  naturally  can  no  longer  be  stimulated  to 
contract. 

In  the  muscles  attacked  by  the  atrophic  process,  unless  the  atrophy 
is  complete,  fibrillary  and  fasciculary  twitchings  very  frequently  appear. 
These  are  occasionally  so  active  in  the  small  muscles  of  the  hand  at 
the  beginning  of  the  disease,  that  they  may  lead  in  the  quietly  resting  hand, 
to  small  jerky  movements  of  the  fingers;  for  instance,  of  the  thumb.  They 
are  especially  striking  in  the  deltoid,  in  which  the  muscular  restlessness  may 
be  increased  to  a  long  and  persistent  wavering  and  oscillation  of  the  individual 
fibres  and  bundles  of  fibres,  which  may  be  still  further  increased  by  stimula- 
tions of  the  skin,  tapping  the  muscle,  under  the  influence  of  cold  and  electric 
stimuli,  as  well  as  by  active  attempts  at  movement. 

The  electrical  excitability  of  the  wasted  muscles,  which  only  in  rare  in- 
stances exhibit  a  tendency  to  fatty  infiltration,  is  quantitatively  always  lowered. 
In  complete  atrophy,  well  localized  tests  in  which  powerful  currents  are 


SPINAL  PROGRESSIVE  MUSCULAR  ATROPHY  303 

employed  and  the  often  very  disturbing  influence  of  a  communicating  loop  of 
the  currents  in  the  muscles  that  are  intact  is  carefully  avoided,  may  demon- 
strate the  presence  of  total  reactions  of  degeneration,  especially  in  the  small 
muscles  of  the  hand.  More  frequently,  however,  the  muscles,  which  are 
usually  considerably  reduced  in  size,  present,  corresponding  to  the  merely 
partial  atrophy,  only  a  -partial  reaction  of  degeneration  with  sluggish  contrac- 
tion upon  direct  galvanic  stimulation. 

Sensibility,  which  should  always  be  thoroughly  tested,  remains  com- 
pletely normal  in  all  its  qualities.  The  paralyzed  arms  and  hands  become 
cyanotic,  as  a  consequence  of  hanging  down  immovably,  and  feel  cold;  the 
skin  over  them  occasionally  becomes  thin  and  delicate,  but  decided  vaso- 
motor and  trophic  changes  are  never  found.  Bladder  disturbances,  disturb- 
ances of  the  genital  functions  and  of  ability  to  empty  the  bowels,  do  not  belong 
to  the  picture  of  the  disease. 

The  occurrence  of  myotonic  phenomena  is  worthy  of  mention  (muscular 
rigidity  after  active  movements,  increased  mechanical  excitability  of  the 
muscles  and  myotonic  electrical  reaction)  in  cases  of  disease  which,  at  least, 
according  to  the  localization  of  the  simultaneously  occurring  muscular  atro- 
phies seem,  very  similar  to  progressive  spinal  myatrophy.  Probably,  it  is  a 
matter,  however,  of  atrophies,  which  proceed  from  the  preceding  myotonic 
change  in  the  muscles  and  are  not  dependent  upon  a  primary  spinal  localiza- 
tion of  the  process. 

Pathological  Anatomy. — Anatomical  examination  in  progressive  mus- 
cular atrophy  reveals  a  wasting  of  the  gray  anterior  horns  in  the  spinal  cord, 
confined  mainly  to  the  cervical  segment.  The  horns  are  reduced  in  size; 
many  ganglion  cells  and  nerve  fibres  disappear.  The  ganglion  cells  still 
present  are  noticeably  changed  in  structure.  The  anterior  roots  are  atrophied 
and  their  medullated  fibres  considerably  diminished  in  number.  In  pure 
cases  the  medullary  sheaths  in  the  spinal  cord  are  intact.  On  the  other  hand, 
there  is  a  loss  of  motor  nerve  fibres  in  the  muscle-nerves  that  belong  to  the 
injured  segments,  and  distinct  changes  of  structure  may  be  seen  to  exist  in  the 
muscles  themselves.  The  muscle  fibres  are  pale  in  muscles  showing  a  high 
degree  of  atrophy  and  show,  in  microscopic  sections,  great  narrowing  and 
proliferation  of  the  nuclei.  A  luxurious  growth  of  connective  tissue  sur- 
rounds the  thin  muscle  fibres,  which  up  to  the  most  extreme  degree  of  atrophy, 
under  otherwise  normal  conditions  still  permit  us  to  recognize  transverse 
stripes,  but  finally  become  reduced  to  very  thin,  slightly  granular,  clouded 
little  fibres,  which  no  longer  show  any  obvious  structure.  Frequently  fibres 
of  considerably  better  preserved  size  and  sharply  defined  structure,  lie  among 
the  muscle  fibres,  which  have  wasted  to  a  considerable  extent  or  even  so 
completely  as  to  leave  the  sarcolemmic  sheath  almost  empty.  The  muscle 
spindles,  corresponding  to  their  physiological  import  as  organs  of  the  deep 
sensibility,  suffer,  in  the  pure  spinal  muscular  atrophy,  no  change. 


3o4  DISEASES  OF  THE  SPINAL  CORD 

A  real  "degenerative"  change  oithe  muscle  fibres  (fatty  degeneration,  wax- 
like changes,  fissures  and  cracks  in  the  muscle  fibres)  does  not  occur  in 
spinal  muscular  atrophy,  which,  as  contrasted  with  myopathic  muscular 
atrophy  shows  only  gradual  anatomical  differences.  Nor  has  the  occurrence 
of  genuine  hypertrophy  of  individual  fibres  within  the  muscle,  which  has  be- 
come seriously  atrophic  in  consequence  of  loss  of  innervation,  been  deter- 
mined with  any  degree  of  certainty.  The  anatomic-histological  examination 
of  the  muscles,  and  especially  of  little  pieces  of  muscle,  taken  from  the  living 
body  or  from  the  dead  body  before  or  during  rigor  mortis  is  extremely  com- 
plicated by  the  changes  occurring  in  the  surviving  contractile  substance 
during  the  preparation,  and  by  the  variable  changes  in  the  condition  of  the 
muscular  protoplasm  dependent  on  metabolism  and  toxic  influences  before 
death.  The  functional  changes  observed  in  the  atrophied  muscles  (electrical 
reaction  of  degeneration)  following  disjunction  of  the  spinal  motor  center  are 
dependent  upon  the  destruction  or  degeneration  of  the  end  branches  of  the 
peripheral  motor  nerves,  not  upon  the  morphological  condition  of  the  muscle 
fibre.  The  term  "degenerative  muscular  atrophy"  which  is  useful,  in  contra- 
distinction to  muscular  atrophy  where  nerve  conduction  is  retained,  is  there- 
fore not  to  be  understood,  in  a  pathologic-anatomical,  but  purely  in  afunctional 
sense,  or  in  relation  to  the  condition  of  the  nerves.  It  concerns  an  atrophy, 
which  goes  hand  in  hand  with  those  changes  of  mechanical  and  electrical 
muscular  excitability,  which  can  be  proved  to  exist  in  "muscles,  the  nerves  of 
•    which  have  been  destroyed." 

The  diagnosis  of  spinal  progressive  muscular  atrophy  is  based  upon  the 
protracted  course  of  the  disease,  extending  over  years  and  decades,  upon  the 
parallelism  between  paralysis  and  muscular  wasting,  upon  the  typical  localiza- 
tion and  the  leap-like  advance  of  the  symptoms,  the  absence  of  the  tendon 
reflexes  and  all  spastic  phenomena  and  the  strict  confinement  of  the  disease 
picture  to  the  motor  sphere.  Especially  does  consideration  of  pains  and  of 
sensory  disturbances  make  it  possible  and,  as  a  rule,  quite  early  in  the  disease, 
to  distinguish  it  from  spinal  gliosis  or  cervical  compression  myelitis;  it  is  in 
respect  to  muscular  changes  alone  that  they  may  give  rise  to  similar  clinical 
pictures.  We  may  refer  here  to  what  was  said  under  amyotrophic  lateral 
sclerosis.  From  this  latter  muscular  atrophy  is  separated  by  the  absence  of 
spasticity  in  the  lower  extremities  and  of  all  reflex  exaggeration.  The  dis- 
tinction from  typical  cases  of  myopathic  dystrophy  is  made  possible  in  most 
cases  by  the  localization  in  the  small  muscles  of  the  hand,  by  the  fibrillary 
twitchings,  and  by  reactions  of  degeneration,  but,  especially  in  respect  to 
localization,  there  are  many  transitional  types.  The  course  of  the  disease 
and  the  succession  of  the  symptoms  allow  us  to  easily  avoid  confusion  with 
multiple  neuritis,  and  with  the  acute  poliomyelitis  of  adults.  Chronic  polio- 
myelitis, which  is  closely  related  to  spinal  muscular  atrophy,  might  furnish 
most  of  the  differential-diagnostic  difficulties,  were  it  not  for  the  fact  that 


SPINAL  PROGRESSIVE  MUSCULAR  ATROPHY  305 

domination  of  the  paralyses  over  the  atrophic  defects,  and  a  broader  exten- 
sion of  the  process  over  larger  masses  of  muscles,  from  the  very  first  speak 
clearly  for  poliomyelitis. 

As  an  infantile  hereditary  and  familial  form  of  progressive  muscular 
atrophy,  cases  of  quickly  progressing  muscular  atrophy  have  been  reported 
Werdnig,  Hoffmann)  which  appear  in  earliest  childhood  in  brothers  and 
sisters,  attack  the  muscles  of  the  thigh,  of  the  pelvic  girdle  and  the  trunk,  then, 
also,  of  the  extremities  and  lead  to  deformity  of  the  vertebral  column.  The 
atrophied  muscles  show  reactions  of  degeneration.  The  children,  in  the  first 
years  of  their  life,  yield  to  the  progressive  muscular  wasting  as  a  result  of 
their  congenital  liability  to  intercurrent  diseases.  The  anatomic  findings 
show  degeneration  of  the  cells  of  the  anterior  horns  and  of  the  peripheral 
nerves. 

The  prognosis  of  spinal  progressive  muscular  atrophy  is  always  un- 
favorable to  this  extent,  that  recovery  or  a  permanent  checking  of  the  disease 
is  impossible;  the  illness  always  shows  a  tendency  to  spread,  even  though 
slowly.  In  this  way  the  condition  of  the  patients  becomes  really  helpless, 
even  if  they  do  not  often  suffer  for  a  long  time  directly  from  the  disease.  A 
dangerous  turn  through  the  appearance  of  bulbar  disturbances,  of  paralysis 
of  respiration,  or  of  intercurrent  diseases,  favored  in  their  effect  by  the  difficulty 
of  the  patients  to  move,  are,  at  all  times  to  be  feared. 

Therapy  is  virtually  powerless  in  face  of  the  progressive  tendency  of  the 
disease.  One  must  be  satisfied  with  avoiding  a  furthering  of  the  progress  of 
the  disease,  by  reduction  of  bodily  movements,  and  guarding  against  all 
over-exertion,  to  which  the  patients,  precisely  because  of  their  weakness, 
are  very  easily  exposed.  Careful  treatment  by  exercises,  arranged  to  suit 
the  peculiarities  of  each  case,  massage  and  the  use  of  the  constant  current 
may  serve  to  strengthen  fibres  still  capable  of  functioning,  in  the  muscles 
that  have  already  been  attacked  and  to  awaken  new  efficiency  in  them. 
It  is  important  to  combat  in  this  way  a  premature  stiffening  of  the  limbs  by  a 
secondary  contracture  of  the  antagonists  (which  have  become  too  powerful) 
by  strychnine  (daily,  subcutaneously  up  to  gr.  1/60)  or  with  injections  in 
the  paralyzed  muscles.  An  attempt  at  medicinal  treatment  by  strychnine 
preparations  administered  in  other  forms,  possibly  combined  with  iron  or 
arsenic,  may  be  recommended.  If  some  suspicious  factors  speak  for  a 
previous  case  of  syphilis  (serum  test,  scars  on  the  skin  or  on  the  mucous 
membranes,  lymphocytosis  of  the  cerebro-spinal  fluid),  a  long  and  energet- 
ically conducted  treatment  with  iodide  of  potassium,  with  or  without  careful 
mercurial  treatment  must  be  attempted,  since,  though  it  cannot  restore  the 
musculature  that  has  been  lost  or  cause  it  to  regenerate,  it  checks,  under 
certain  conditions,  further  development  of  the  disease,  or  of  other  nervous 
maniiestations  thereof. 


306  DISEASES  OF  THE  SPIXAL  CORD 

(f)  Neuritic  Progressive  Muscular  Atrophy 

Peroneal,  Forearm  Type  of  Progressive  Muscular  Atrophy 
{Char cot-Marie,  Hoffmann) 

BY 

FR.  JAMIN  (Erlangen) 

Among  the  many  and  various  forms,  in  which  systemic  change  of  the  nerv- 
ous system  may  express  itself  preeminently  by  myoatrophic  conditions,  a 
group  of  diseases,  not  very  frequently  observed,  may  be  set  aside  from  the 
closely  related  pictures  of  spinal  and  pure  myopathic  atrophies,  which  usually, 
if  not  always,  appear  familially  and  in  childhood  are  characterized  by  the 
localization  of  degenerative  muscular  atrophies  in  the  distal  parts  of  the 
body,  in  the  foot  and  leg,  the  hand  and  the  forearm,  and  by  slight  disturb- 
ances of  sensation  prove  the  participation  of  the  sensory  conducting  paths; 
in  most  cases  these  changes  predominate  in  the  peripheral  nerves. 

This  neuritic  progressive  muscular  atrophy  begins  very  gradually  in 
later  childhood,  in  some  cases,  however,  not  until  adult  life.  It  attacks,  as 
a  rule,  several  members  of  a  family,  sometimes  through  several  genera- 
tions, sparing  neither  boys  nor  girls,  but  showing  a  distinct  preference 
for  the  former.  Isolated  cases  occur  as  well.  If  the  disease  has  attacked  a 
family,  it  may  skip  individuals  or  entire  generations,  only  to  reappear 
among  their  descendants. 

The  disease,  then,  is  decidedly  endogenous,  developing  upon  congenital 
foundations.  Accidental  etiological  factors — getting  drenched,  and  catching 
cold,  traumatism,  post-infectious  cachexias  and  intoxications — may  be  said  to 
be  only  exciting  causes  of  the  disease,  or  to  assist  in  its  development. 

The  arrangement  of  the  symptoms,  in  particular  the  localization  of  the 
myoatrophy,  is  very  characteristic.  The  patients  themselves  become  aware 
of  their  disease  by  the  remarkable  weariness  and  weakness  of  the  feet  in 
walking,  and  also  by  the  change  in  the  contour  of  the  feet,  which  interferes 
with  the  use  of  shoes. 

Paresis  and  circumscribed  muscular  atrophy,  discovered  in  the  examina- 
tion, appear  first  in  the  small  muscles  of  the  foot;  they  cause  the  contours  of 
the  bones  upon  the  dorsum  to  stand  out,  the  sole  is  deepened,  and  the  medial 
edge  of  the  foot  is  flattened  out.  Early,  too,  appear  paralysis  and  atrophy 
of  the  muscles  of  the  leg  that  are  supplied  by  the  nervus  peroneus,  above  all, 
of  the  extensor  hallucis  longus,  then,  too,  of  the  extensor  digitorum  and  of  the 
peronei,  also  of  the  tibialis  anticus.  The  anterior  and  external  sides  of  the  leg, 
therefore,  appear  emaciated  and  through  this  atrophy  raising  the  foot, 
especially  its  outer  edge,  becomes  impossible,  likewise  dorsal  flexion  of  the 
toes,  above  all  of  the  great  toes.  When  the  leg  is  raised,  the  tip  of  the  foot, 
therefore,  as  in  peroneus  paralysis,  drops  towards  the  floor  with  the  claw-like 


NEURITIC  PROGRESSIVE  MUSCULAR  ATROPHY 


3°7 


toes  in  plantar  flexion.  In  the  beginning,  and  occasionally  for  a  considerable 
period  of  time,  the  foot  remains  easily,  even  abnormally  movable  at  the 
ankle-joint;  it  swings  loosely  in  the  ankle-joint  in  passive  movements.  But 
since,  as  time  goes  on,  the  contrast  in  efficiency  of  the  atrophied  extensors  of 
the  foot  and  toes  and  the  considerably  better  conserved  muscles  on  the  flexor 
side  of  the  leg,  of  the  group  of  the  calf  muscles,  becomes  more  and  more 
pronounced,  contractures  form  (cf.  Fig.  69).  There  develops,  symmetrically 
on  both  sides,  a  formation  of  pointed  and  club  foot,  that  can  no  longer  be 
made  normal,  a  pes  equinus  varus.  Since  in  the  course  of  the  disease,  the 
triceps  surae  is  usually  also  (gastroc- 
nemius and  soleus)  attacked  by  the 
atrophic  process,  so  that  the  curve  of 
the  calf  is  lost,  the  plantar  flexion  of 
the  foot  may  also  be  lost;  the  con- 
tracture position  is  then  controlled  by 
the  long  flexors  of  the  toes  and  the 
tibialis  posticus,  which  often  continues 
powerful  for  a  very  long  time;  when 
the  action  of  the  peroneal  muscles  is 
completely  lost,  it  causes  the  foot  to 
turn  inward  with  the  formation  of 
"hollow  foot."  Then  the  toes  can  no 
longer  be  passively  extended.  The 
patients  now  walk  upon  the  outer 
edge  of  the  foot,  even  upon  the  outer 
dorsal  side  of  the  foot,  upon  which 
callus  forms,  and  since  the  patients  are 
mostly  young  individuals,  the  tarsal 
bones  may  be  crowded  out  and  moved 
from  their  natural  places. 

The  muscles  of  the  thigh  become  atrophic  late  and  to  a  lesser  degree. 
On  the  other  hand,  there  appears  earlier,  in  rare  cases,  even  preceding  the 
paralysis  in  the  feet,  an  atrophy  and  corresponding  paralysis  of  the  small 
muscles  of  the  hand  and  the  extensors  of  the  forearm  with  claw-like  formation  of 
the  hands  and  tapering  of  the  distal  division  of  the  arm,  as  it  is  described 
in  spinal  muscular  atrophy.  In  later  stages  of  the  disease,  the  muscles  of  the 
upper  arm  and  shoulder  participate  also,  to  a  moderate  degree,  in  the  mus- 
cular wasting,  whereas  the  muscles  of  the  face  and  the  trunk,  also  the  bulbar 
realms  are  mostly  spared,  and  therefore  the  muscular  atrophy  in  these  cases 
rarely  reaches  such  proportions  as  to  seriously  threaten  health  and  life. 

The  atrophied  muscles  show  occasionally,  but  not  regularly,  fibrillary 
twitchings.  Their  electrical  excitability  is,  both  upon  indirect  and  direct 
stimulation,^  lowered  quantitatively,   corresponding   to   thej_degree   of   the 


fck^l 

^L         ^r 

wS\ 

^.        1 

H 

1          9 

I           J 

^A       SI 

mm          £ 

Fig.  69. — Posture  of  the  feet  in  neuritic  mus- 
cular atrophy.     (Erlangen  Medical  Clinic.) 


308  DISEASES  OF  THE  SPINAL  CORD 

atrophy;  under  certain  conditions  it  is  entirely  lost.  In  the  regions  that 
are  more  severely  affected,  the  peroneal  muscles,  the  small  muscles  of  the 
hand,  etc.,  the  presence  of  partial  or  total  reactions  of  degeneration  may  be 
demonstrated. 

The  tendon  and  periosteal  reflexes  are,  always,  so  far  as  the  atrophy  extends, 
frequently,  too,  throughout  the  extremities,  wholly  lost  or  considerably  weak- 
ened. The  cutaneous  reflexes  are  not  changed,  in  so  far  as  they  are  not 
necessarily  restricted  by  the  loss  of  muscular  regions  that  have  become  atro- 
phic, or  by  the  contractures. 

Sensibility  is  really  not  much  disturbed,  but  in  many  cases  it  does  not 
remain  perfectly  normal;  painful  sensations  and  paresthesias  are  observed 
in  the  atrophic  limbs,  which  can  not  be  explained  by  the  changes  of  position 
and  movement  alone,  also  dulling  of  the  superficial  sensibility  to  the  point  of 
complete  anaesthesia  in  the  feet.  The  feet  are  cyanotic  and  feel  cool.  More 
serious  trophic  disturbances  do  not  develop.  Bladder  and  rectal  disturbances 
are  also  absent. 

The  pathologic-anatomic  investigations  have  shown  that  this  disease  is 
dependent  upon  degenerative  changes  in  the  peripheral  nerves,  which  supply 
the  atrophied  muscles,  especially  in  their  distal  intramuscular  ramifications. 
Atrophies  in  the  corresponding  segments  of  the  gray  anterior  columns  of  the 
spinal  cord  have  also  been  found.  Likewise  the  posterior  columns  (funiculi 
graciles  Goll)  have  been  found  to  be  the  seat  of  chronic  changes.  The 
changes  in  the  atrophied  muscles  correspond  to  the  picture  outlined  in  spinal 
muscular  atrophy — serious  muscular  wasting  with  connective  tissue  changes  of 
the  muscle  parenchyma,  while  fatty  infiltration  is  rarely  present,  and  genuine 
muscular  hypertrophy  occurs  but  seldom  in  the  well-preserved  muscles,  which 
in  part  are  strengthened  through  their  compensatory  activity. 

The  diagnosis  is  based  on  the  beginning  of  the  disease  in  childhood,  the 
very  protracted  course,  the  peculiar  localization  of  the  symptomatic  muscular 
wasting,  which  resembles  spinal  muscular  atrophy  of  the  peroneal-forearm 
type.  An  absolute  strict  differentiation  from  spinal  muscular  atrophy  can 
not  always  be  made  because  of  the  anatomic  changes  which  in  many  respects 
are  identical,  and  there  are  transitions  even  to  myopathic  dystrophy,  which, 
however,  as  a  rule,  begins  from  adjacent  groups  of  muscles.  Genuine 
polyneuritis  develops  far  more  quickly  into  a  paralysis,  which  has  occasion- 
ally similar  localization,  but  is  usually  far  more  extended,  displays  more 
striking  sensory  disturbances  (pains,  ataxia)  and,  after  a  stormy  course, 
permits  the  return  of  ability  to  move,  while  neuritic  muscular  atrophy,  in  its 
symmetrical,  gradually  progressing  extension,  produces  irreparable  defects. 
The  continuous  increase  in  the  wasting  of  muscles,  lasting  for  years,  from 
slight  beginning  in  the  periphery,  distinguishes  it  from  poliomyelitis  also. 

A  disease  similar  to  this,  with  similarly  localized  muscular  wasting,  the 
same  disturbances  of  electrical  excitability,  and  slight  sensory  disturbances, 


NEURITIC  PROGRESSIVE  MUSCULAR  ATROPHY 


3°9 


has  been  described  under  the  name  interstitial  hypertrophic  progressive 
neuritis;  this  disease  may  also  be  familial.  It  is  distinguished  from  the 
peroneal  form  of  muscular  atrophy  by  the  objectively  demonstrable  thicken- 
ings of  the  peripheral  nerves  (peroneus,  ulnaris)  as  well  as  by  the  complica- 
tion with  distinct  disturbances  in  co-ordination. 


Fig.  70. — Neuritic  muscular  atrophy.     Pronounced    atrophy  of    the   peripheral    parts    of   the 
extremities,  especially  in  the  leg  proper.     (After  Schoenborn  and  Krieger.) 

The  prognosis  with  regard  to  life  is  not  really  unfavorable.  The 
ability  to  move  and  the  possibility  of  following  one's  vocation,  are,  however, 
always  to  a  great  extent  impaired,  especially  when  the  upper  extremities  also 
are  severely  attacked. 

Therapy  cannot  stop  the  wasting  of  the  muscles.     By  massage   and 


3io  DISEASES  OF  THE  SPINAL  CORD 

electrical  treatment,  as  well  as  by  a  diligent  care  of  the  entire  body,  the  rate  of 
the  progression  may  be  somewhat  influenced,  and  the  condition  of  the  patient 
is  made  distinctly  more  endurable  by  a  careful  strengthening  of  the  retained 
muscles  by  exercise  and  use.  The  changes  in  form  and  posture  of  the  feet, 
which  are  most  disturbing,  cannot  be  removed  permanently  or  checked  by 
supporting  apparatus.  The  function  of  the  preserved  muscles,  especially  of 
the  tibialis  posticus,  always  forces  the  foot,  if  it  is  to  be  used  at  all,  into  an 
abnormal  position  again.  As  in  all  progressive  muscular  atrophies,  plastic 
operations  also  afford  little  prospect  of  permanent  results.  The  gait  and 
posture  of  the  patient  may  be  best  improved,  by  first  loosening  the  tendons 
shortened  by  contracture,  and  then  anchylosing  the  ankle-joint  into  a  correct 
position  permanently  by  an  operation  (arthrodesis) ,  in  order  that  the  action  of 
the  better  preserved  muscles  of  the  pelvis  and  the  thigh  may  be  given  a  safer 
support  in  walking. 

(g)  Subacute  and  Chronic  Poliomyelitis 

BY 

FR.  JAMIN  (Erlangen) 

In  rare  cases,  in  adults,  a  disease  is  observed,  strictly  confined  to  the 
peripheral  motor  system,  associated  with  muscular  wasting,  which,  in  the 
localization  of  the  paralysis  and  the  muscular  atrophy,  as  in  the  developing 
sequence  of  the  phenomena,  can  be  distinguished  from  spinal  progressive 
muscular  atrophy,  as  well  as  from  the  acute  inflammatory  diseases  of  the 
anterior  columns  and  the  peripheral  motor  nerves. 

Probably  subacute  and  chronic  inflammatory  processes  are  concerned, 
together  with  degeneration  and  atrophy  in  the  gray  anterior  columns  of  the 
spinal  cord,  which  owe  their  origin  to  the  selective  effect  of  chronic  poisonings. 
Lead  poisonings,  autointoxications,  diabetes  mellitus  are  considered  causal 
factors.  It  is  possible  that  traumatic  shocks  favor  the  peculiar  localization 
of  the  process  of  the  disease.  In  the  pathologic-anatomical  picture,  chronic, 
inflammatory  changes  in  the  vessels,  as  well  as  extensive  degenerative- 
atrophic  changes  in  the  ganglion  cells  and  in  the  mass  of  fibres  of  the  anterior 
horns,  have  been  demonstrated. 

In  the  clinical  picture  the  symptoms  of  paralysis  are  most  promi- 
nent. Immediately,  without  any  signs  of  a  general  disease,  there  usually 
appears  first  a  weakness  in  the  legs,  which  is  increased  in  the  course  of  a  few 
days  to  a  widespread  paralysis  of  the  extremities,  but  which,  nevertheless, 
usually  remains  incomplete,  attacking  some  groups  of  muscles  more  severely, 
and  sparing  others.  The  extent  of  the  paralysis  is  not  confined  within  definite 
bounds.  In  irregular  succession  one  limb  after  another  may  be  attacked,  or 
both  legs  may  be  paralyzed  alone,  or  both  arms.  After  weeks,  and  months 
in  some  cases,  all  four  extremities  may  have  become  paralytic,  nor  do  the 


SUBACUTE  AND  CHRONIC  POLIOMYELITIS 


3" 


muscles  of  the  trunk  remain  intact;  and  in  the  decidedly  progressive  cases, 
there  finally  appears  a  paralysis  of  a  bulbar  character,  which  together  with 
a  paralysis  of  the  muscles  of  respiration  may  lead  to  death. 

Only  after  the  serious  motor  functional  disturbance  has  been  present  for 
some  time,  does  the  "degenerative"  muscular  wasting  become  apparent  in  the 


Fig.  71. — Poliomyelitis  anterior  chronica.     Severe  atrophic  paralysis  of  both  hands;  at  the  left 
side  also  atrophy  of  the  deltoideus  and  the  brachio-radialis.     (After  Schoenborn  and  Krieger.) 

paralyzed  regions.  The  muscles  decrease  in  volume  quickly  and  to  a  consid- 
erable degree,  become  limp  and  weak.  As  a  rule  they  show  fibrillary  twitchings. 
The  electric  examination  permits  us  to  recognize  a  diminution  of  the  electrical 
excitability  and  partial  or  total  reactions  of  degeneration,  even  in  paralyzed, 
but  as  yet  slightly  atrophied,  muscles.     The  tendon  reflexes  are  markedly 


3i2  DISEASES  OF  THE  SPINAL  CORD 

diminished  or  entirely  abolished,  and  the  cutaneous  reflexes  are  lowered  to  a 
degree  corresponding  to  the  paralysis. 

The  sensibility  in  the  pure  cases,  remains  intact  in  all  its  qualities,  should 
there  be  no  more  diffuse  injury  of  the  spinal  cord.  In  particular,  stronger 
sensory  phenomena  of  irritation,  also  trophic  disturbances,  and  disturbances 
in  the  urogenital  and  intestinal  innervation  are  absent. 

The  disease  does  not  always  progress  to  paralysis  of  all  four  extremities, 
the  trunk  and  the  motor  cerebral  nerves.  After  weeks,  it  may  become  sta- 
tionary, with  more  or  less  extensive  paralytic  and  atrophic  defects  remaining 
non-retrogressive,  or,  after  months,  there  may  possibly  be  an  extensive  im- 
provement of  the  condition,  even  recovery. 

In  regard  to  differential  diagnosis,  the  common  polyneuritis  is  of 
importance,  since  it  has  caused  confusion  with  chronic  poliomyelitis.  But 
it  is  distinguished  from  this  by  the  course,  which  is  mostly  more  rapid,  and 
above  all,  by  the  appearance  of  sensor}7  phenomena  of  irritation  and  of  loss 
of  function,  severe  plains,  sensitiveness  of  the  limbs  and  the  nerve  trunks  to 
pressure,  and  disturbances  of  sensibility  and  ataxia.  In  spinal  muscular 
atrophy,  and  neuritic  muscular  atrophy,  there  develop  the  more  sharply 
circumscribed  functional  disturbances,  corresponding  directly  to  the  degree 
of  the  muscular  wasting,  and  progressing  simultaneously  from  fibre  to 
fibre  with  it.  They  progress  far  more  slowly  than  chronic  poliomyelitis, 
which  in  quick  succession,  at  once  paralyzes  entire  groups  of  muscles. 

The  prognosis  may  be  doubtful  in  the  beginning  of  the  paralysis.  If 
the  functional  disturbance  soon  remains  confined  to  a  smaller  region,  and, 
after  the  development  of  the  atrophy  only  partial  reaction  of  degeneration 
sets  in  (middle  form)  the  chances  for  recovery  are  not  unfavorable.  But 
if  prolonged  investigation  shows  that  the  flaccid  atrophic  paralysis  marches 
on  and  on  and  can  not  be  checked,  it  is  to  be  feared  that  the  vital  muscular 
regions  will  be  affected,  and  that  the  disease  will  be  fatal. 

The  treatment  in  the  advanced  cases  must  be  guided  by  the  principles 
that  hold  good  for  acute  poliomyelitis  and  spinal  muscular  atrophy.  At 
the  beginning  of  the  paralysis,  rest  in  bed  and  very  little  exercise  of  the 
muscles  is  suggested;  massage  too  is  to  be  applied  only  very  cautiously; 
in  any  case  galvanic  treatment  with  the  anode,  whose  action  is  not  so  ener- 
getic, is  to  be  preferred  to  the  use  of  faradic  currents.  Only  when  the 
phenomena  retrogress  noticeably,  may  the  regeneration  of  the  nerves  and 
muscles  be  furthered  by  persistent  methodical  treatment  with  the  constant 
current.  Where  there  is  some  suspicion  of  a  toxic  aetiology,  one  should 
endeavor  to  eradicate  it  by  dietetic  measures,  sweating  and  baths,  also  by 
medicines  (iodide  of  potassium,  preparation  of  salicylic  acid)  with  exact 
control  over  the  excretions.  The  possibility  that  similar  forms  of  disease 
may  develop  after  a  syphilitic  infection  ought  to  be  considered  in  the 
treatment. 


\COMBINED  SYSTEM  DISEASES  OF  THE  SPINAL  CORD     313 
(h)  The  Combined  System  Diseases  of  the  Spinal  Cord 

BY 

FR.  JAMIN  (Erlangen) 

The  detailed  anatomic  investigation  of  the  endogenous,  congenital, 
primary  column  diseases  of  the  spinal  cord,  which  are  characterized  by 
hereditary  and  familial  occurrence  (Friedreich's  ataxia,  spastic  spinal 
paralysis)  showed  that  not  infrequently,  a  combined  lesion  of  several  systems 
in  the  lateral  and  posterior  columns  of  the  spinal  cord  proved  to  be  present, 
even  though  the  clinical  picture  of  the  disease  was  distinguished  by  the 
predominance  of  functional  disturbance  in  one  system  group;  in  spastic 
paralysis,  of  the  pyramidal  tracts;  in  Friedreich' s  ataxia,  of  the  posterior 
columns  and  the  cerebellar  lateral  columns.  It  was  natural,  therefore,  to 
assume  that  pictures  of  symptoms,  which  allow  us  to  conjecture  the  presence 
of  an  approximately  similar  functional  disturbance  in  the  various  sensory 
and  motor  tracts  of  the  spinal  cord,  have  also  been  caused  by  a  primary 
selective  injury  -in  the  fibre  systems  of  the  posterior  and  lateral  columns, 
whether  this  injury  be  based  upon  a  peculiar  inborn  weakness  of  this  system, 
relative  to  its  being  used  up  easily,  or  to  general  noxse,  or  whether  it  is  caused 
by  the  specific  effect  of  toxic  influences  on  these  systems  of  tracts.  As  a 
matter  of  fact,  there  are  a  series  of  observations,  in  which  the  clinical  picture 
of  the  disease  permits  us  to  recognize  almost  exclusively  a  functional  dis- 
turbance of  the  long  tracts  of  the  spinal  cord,  especially  of  the  pyramidal 
anterior  columns,  the  pyramidal  lateral  columns,  the  posterior  columns, 
and  the  cerebellar  lateral  columns;  and  the  anatomical  findings  also  seem  to 
establish  the  assumption  of  a  pure  primary  combined  columnar  disease. 
But  if  the  undoubtedly  primary  endogenous  columnar  diseases,  which  are 
discussed  in  the  chapters  on  Friedreich's  ataxia,  and  spastic  spinal  paralysis, 
are  left  out  of  consideration  and  also  the  many  varied  combinations  of  tabetic 
phenomena  as  being  an  exogenous  disease,  the  pathogenetic  relations  for 
the  isolated  cases  of  clinically  demonstrable  combined  columnar  diseases, 
are  found  considerably  more  complicated. 

Pathological  Anatomy. — The  more  recent  investigations  by  Nonne 
and  Frnend,  Henneberg  and  other  authors,  have  shown  that  in  most  of  the 
cases,  the  detectable  degenerations  in  the  spinal  cord  correspond,  on  the 
whole,  to  the  course  of  the  long  fibre  systems  of  the  pyramidal  tracts,  the 
posterior  columns,  and  the  lateral  cerebellar  tracts,  but  that  they  are  not 
strictly  confined  to  these  systems,  but  spare,  in  part,  definite  sections, 
especially  those  directly  adjacent  to  the  gray  substance  and  partly  extend 
irregularly  at  various  levels  of  the  spinal  cord  in  various  ways,  and  often 
asymmetrically,  beyond  the  area  of  the  well-known  tracts.  These  variations 
in  the  spreading  of  the  degeneration  over  the  cross-section  of  the  individual 
column  systems,  cannot  be  quite  satisfactorily  explained  by  the  individual 


3i4  DISEASES  OF  THE  SPINAL  CORD 

differences  in  the  development  of  the  physiologically  differing  systems. 
In  addition,  transitional  types,  from  apparently  purely  systemic  columnar 
degeneration  to  decidedly  focal  disease  of  the  spinal  cord,  occur. 

In  those  places,  in  which  the  degeneration  in  the  posterior  and  lateral 
columns  is  most  marked,  in  the  dorsal  or  cervical  segments,  dense  sclerotic 
glia  growths  appear,  in  place  of  the  degenerated  posterior  columns  and  in 
the  center  of  the  lost  pyramidal  lateral  column,  whereas  the  similarly 
destroyed  lateral  cerebellar  tracts  are  replaced  by  a  looser  net-work  of  glia 
fibres.  In  the  midst  of  the  scleroses,  however,  thickenings,  hyaline  degenera- 
tions and  perivascular  infiltrations  or  scleroses  are  found  on  the  vessels,  which 
seem  to  confirm  the  supposition  that  it  is  not  a  matter  of  primary  degeneration 
of  the  columnar  fibres,  but  of  the  cicatricial  end-result  of  focal  diseases  of  the 
white  substance  emanating  from  the  vascular  system.  By  the  confluence  of 
small  focal  lesions  in  the  white  matter,  there  arises,  associated  with  the 
phenomena  of  ascending  and  descending  degeneration,  from  the  picture  of  a 
disseminated  myelitis  the  picture  of  a  visible,  but  by  no  means  strictly 
confined  columnar  disease.  For  the  peculiar  arrangement  of  the  focal 
disease  the  white  matter's  scant  supply  of  blood-vessels  from  the  small 
branches  of  the  marginal  arteries  of  the  spinal  cord  is  the  cause,  in  the 
area  of  which,  naturally,  with  slighter  disturbances  in  circulation,  sclerosis 
of  the  fines  tramifications  and  similar  injuries  appears  more  easily  than  in 
the  far  more  richly  supplied  gray  matter.  Nevertheless  here,  too,  as  in 
other  injuries  which  affect  the  cross-section  of  the  spinal  cord  over  a  con- 
siderable space,  the  increased  vulnerability  of  the  long  paths,  especially 
of  the  posterior  columns,  and  the  pyramidal  tracts  may  influence  the  arrange- 
ment of  the  degenerations,  and  especially  the  distributions  of  the  functional 
disturbances.  It  is  to  be  considered  also  in  comparing  the  anatomical 
findings  with  the  clinical  picture  of  the  disease,  that  the  anatomic  picture, 
especially  in  so  far  as  it  is  confined  to  the  degeneration  of  the  medullated 
sheaths  alone,  and  does  not  affect  the  make  up  of  the  axis  cylinders,  is  not 
able  to  fully  explain  the  functional  disturbances.  This  explains  also  why 
under  certain  conditions,  when  there  are  extensive  anatomic  changes  in  the 
spinal  cord,  only  slight  functional  disturbances  are  observed,  and  vice  versa. 
The  lateral  cerebellar  tracts  only  participate  systemicly  in  the  pathological 
process,  if  the  degeneration  of  the  lateral  columns  in  the  upper  dorsal  portion 
of  4the  cord  is  considerable,  because,  in  that  case,  the  fibres  passing  from 
Clarke's  columns  to  the  lateral  column  are  injured  on  their  way  through  the 
focal  injury  in  the  lateral  column.  Clarke's  columns,  themselves,  are  often 
found  intact;  in  other  cases,  they  too  have  become  atrophic,  probably 
through  retrograde  degeneration. 

If,  therefore,  it  is  probable,  that  the  combined  columnar  diseases  are 
caused,  not  by  a  primary  selective  injury  of  the  tracts,  but  by  disseminated 
focal  disease  as  a  consequence  of  disturbances  in  circulation,  ischasmia,  or 


COMBINED  SYSTEM  DISEASES  OF  THE  SPINAL  CORD      315 

by  an  injury  in  the  white  matter,  the  result  of  a  blood-vessel  dis- 
turbance, yet  in  many  cases,  the  preference  for  the  posterior  columns,  the 
pyramidal  tracts,  and  the  lateral  cerebellar  tracts  gives  these  processes  of 
degeneration  a  peculiar  impress,  and  justifies  us  in  regarding  these  forms  of 
disease,  which  differ  even  in  the  clinical  course  from  the  acute  myelitic 
processes,  as  a  separate  group. 

iEtiology. — The  causes  of  the  pathological  changes  described,  which 
are  rarely  examined  anatomically  until  the  stage  of  the  secondary  and  the 
resulting  cicatricial  conditions,  are  manifold.  From  a  number  of  infectious 
and  toxic  injuries;  from  septic,  chronic  nephritic  and  tubercular  processes, 
from  pellagra,  alcoholism,  diabetes,  carcinomatosis,  lead  poisoning,  com- 
bined columnar  diseases  may  result.  Syphilis,  too,  may  evoke  a  similar 
disease  picture..  With  special  frequency,  extensive  lesions  seem  to  develop, 
in  serious  anaemic  and  cachectic  conditions  such  as  in  pernicious  anaemia, 
and  leucaemia.  In  many  of  these  constitutional  diseases,  special  importance 
attaches  probably  to  the  disturbances  of  circulation,  caused  by  arterioscle- 
rotic changes  in  the  smallest  vessels,  and  it  is  probable,  that  trauma- 
tisms, with  general  concussion  to  the  spinal  cord,  can  exercise  an  exciting 
influence. 

Symptomatology. — Depending  upon  the  cause  underlying  the  disease, 
and  the  extent  of  the  pathological  process  in  the  spinal  cord,  i.  e.,  of  the 
functional  injuries  caused  thereby,  the  disease  picture  of  combined  system 
disease  may  develop  in  various  ways.  The  course  of  the  disease  is  not 
rarely  very  acute,  with  rapid  development  of  far-reaching  spinal  functional 
disturbances;  in  other  cases,  the  disease  shows  rather  a  subacute  course, 
changing  in  the  form  of  its  symptoms  with  the  spread  of  the  changes  to 
various  levels  of  the  white  matter,  and  in  the  cross-section  of  the  white  fibre 
system.  A  chronic,  quite  slowly  progressing  form  of  the  symptom  complex 
has  also  been  reported.  In  the  beginning  of  the  disease  at  times  there  is 
nothing  more  noticeable  than  slight  subjective  disturbances  of  feeling, 
paresthesias:  probably,  in  other  cases  also,  slight  pains  of  the  character  of 
irritation  symptoms  of  the  roots  as  well,  and  there  are  cases  in  which  more 
important  objectively  detectable  spinal  symptoms  of  functional  loss  are 
never  developed.  But  in  most  instances,  the  system  degenerations  lead  to 
clearly  recognizable  functional  disturbances  in  the  lateral  columns,  pre- 
eminently in  the  pyramidal  tracts  on  the  one  side,  and  the  posterior  columns, 
probably  with  the  inclusion  of  the  radiation  areas  of  the  posterior  roots  on 
the  other  side.  To  these  are  added  eventually  the  phenomena  of  functional 
loss,  which  are  dependent  upon  a  participation  of  the  lateral  cerebellar 
tracts  and  the  antero-lateral  columns. 

Preeminently  two  groups  of  symptoms  appear  concurrently  as  they  appear 
in  tabes  and  Friedreich's  disease  for  the  posterior  column  affection,  in  spastic 
spinal  paralysis  for  the  lateral  column  affection.     By  the  union  of  both,  or 


316  DISEASES  OF  THE  SPINAL  CORD 

an  overlapping  of  the  groups  of  symptoms,  there  arise  various  types  of  com- 
bined system  disease  (Oppenheim,  E.  Mutter) .  Degeneration  of  the  posterior 
column  alone  leads  to  the  loss  of  the  deep  sensibility,  to  ataxia,  to  hypotonia  of 
the  limbs  and  to  the  loss  of  the  tendon  reflexes.  The  degeneration  of  the 
■lateral  pyramidal  tracts  causes  paresis  of  the  extremities,  especially  in  the  legs 
with  predilection  for  the  flexors,  synergisms  (tibialis  phenomenon,  etc.) ,  after 
longer  duration  a  tendency  to  contractures,  changes  of  the  cutaneous  reflexes 
(Babinski,  Oppenheim),  exaggeration  of  the  tendon  reflexes,  and  particularly 
to  hypertonia  of  the  muscles.  If  both  systemic  diseases  occur  together,  the 
symptoms  must,  in  part,  mutually  neutralize  each  other,  according  as  the 
affection  of  the  posterior  columns  or  that  of  the  lateral  columns  gradually 
and  according  to  their  special  expansion  preponderate.  If,  as  is  frequently 
the  case,  the  functional  lesion  of  the  pyramidal  tracts  predominates  in  the 
clinical  picture,  the  symptoms  of  hypertonia,  of  spastic  paraparesis,  usually, 
since  it  is  a  matter  of  disturbance  in  the  dorsal  segment,  in  the  legs  alone, 
with  exaggeration  of  the  tendon  reflexes,  synergisms  and  changes  of  the 
cutaneous  reflexes  step  into  the  foreground,  while  disease  of  the  posterior 
columns  makes  itself  felt  only  by  slight  disturbances  of  the  deep  sensibility  in 
the, distal  sections  of  the  extremities,  as  well  as  by  ataxic  disturbances  in 
movement,  which  may  be  increased  upon  participation  of  the  cerebellar 
lateral  tracts  by  the  phenomena  of  static  ataxia.  Then  we  have  the  fre- 
quently observed  symptom  complex  of  spastic  ataxic  paraparesis. 

If,  on  the  other  hand,  the  injury  to  the  posterior  column  predominates, 
or  gradually  overshadows  the  lateral  column  lesion,  the  disease  picture 
assumes  a  more  tabetic  character.  The  ataxia  makes  itself  felt  in  a  more 
considerable  degree,  the  sensibility  disturbances  become  more  prominent,  the 
tendon  reflexes  disappear.  At  times,  even  the  paresis  of  the  lower  extremities 
becomes  flaccid,  the  muscles  become  atonic.  In  most  of  these  cases,  however, 
some  degree  of  hypertonia  (while  tendon  reflexes  are  lost)  persists  as  a  sign  of 
the  simultaneously  present  lateral  column  lesion;  this,  beside  the  change  of 
the  cutaneous  reflexes  (Babinski,  etc.),  beside  a  slight  paresis  of  the  predi- 
lection type  and  characteristic  changes  in  the  posture  of  the  legs,  which  finally 
pass  over  into  contractures  (dorsal  flexion  of  the  great  toe,  supination  of  the 
foot  in  the  equinus  position)  permits  us  to  conclude,  that  there  is  participation 
of  the  pyramidal  tracts  in  the  process  of  the  disease. 

More  extensive  disturbances  in  sensation,  even  in  the  realm  of  superficial 
sensation,  suggest  a  participation  of  the  tracts  of  the  antero-lateral  columns. 
It  is  worthy  of  note  that  the  sensory  disturbances  in  combined  system  diseases 
of  the  above-mentioned,  more  pseudosystemic  form,  are  occasionally  sharply 
limited  upward,  and  so  can  simulate  a  pure  cross-section  lesion  (Nonne, 
Frund) .  To  the  realm  of  the  affections  of  the  posterior  columns  in  the  wider 
sense  belong  the  bladder  disturbances,  which  are  very  frequently  observed  in 
cases  belonging  to  this  group:  retention  of  the  urine,  frequently  insurmount- 


COMBINED  SYSTEM  DISEASES  OF  THE  SPINAL  CORD       317 

able  vesical  tenesmus,  finally  loss  of  the  voluntary  regulation  of  the  bladder 
function.    Rectal  disturbances,  retention  of  the  feces,  coprostasis  occur  also. 

In  rare. cases,  the  degenerative  process  does  not  stop,  continuing  even  to 
the  higher  bulbar  divisions  of  the  central  nervous  system  and  leads  to  the 
phenomenon  of  obsessional  crying  and  laughing  appertaining  to  the  spastic 
symptom  complex,  to  dysarthria,  nystagmus,  vertigo,  and  a  high  degree  of 
cerebellar  ataxia. 

The  realms  of  the  sensory  cerebral  nerves  do  not,  as  a  rule,  participate  in 
the  process  of  the  disease.  Several  times  a  neuritis  optica  has  been  observed 
to  exist  in  the  combined  system  disease  analogously  to  certain  forms  of 
disseminated  myelitis.  The  pupillary  phenomena  are  not  changed  or 
influenced  in  the  former. 

The  diagnosis  of  combined  column  disease  is  difficult  and  only  to  be 
made  very  cautiously.  It  can  be  based  upon  proof  of  the  existence  of  the 
combination  of  posterior  column  symptoms  with  lateral  column  symptoms, 
when  either  the  tabetic  or  the  spastic  symptom  complex  predominates,  when 
signs  are  wholly  lacking  of  a  focal  injury  in  the  gray  matter  of  the  spinal  cord 
(dissociated  disturbances  in  sensation,  degenerative  paralyses  and  localized 
muscular  atrophies)  and  upon  the  absence  of  symptoms  of  irritation  pertain- 
ing to  the  posterior  roots  (relatively  violent  pains).  The  course  of  the  dis- 
ease, which  usually  is  free  from  suddenly  appearing  exacerbations,  is  also,  to 
a  certain  extent,  a  characteristic  feature.  It  is  to  be  remembered  that  in 
tabes  likewise  there  often  appears  a  combined  system  disease,  a  participation 
of  the  lateral  columns,  and  in  progressive  paralysis  as  well;  the  existence  of 
pupillary  disturbances,  lancinating  pains,  examination  of  the  cerebro-spinal 
fluid  will  permit  us  to  recognize  under  certain  conditions,  the  tabetic  nature 
of  the  changes.  Furthermore,  all  those  spinal  diseases,  which  cause  a  func- 
tional disturbance  of  the  posterior  columns  and  the  lateral  columns  to  ap- 
pear through  disseminated  disturbances  or  a  slighter  cross-section  lesion,  as 
cerebro-spinal  syphilis,  which  is  characterized  by  relatively  violent  pains  and, 
occasionally,  by  early  appearing  peripheral  paralyses,  compression  of  the 
spinal  cord,  spinal  gliosis,  etc.,  are  to  be  excluded  before  one  is  justified  in 
thinking  of  a  combined  system  disease.  Differentiation  from  the  spastic 
ataxic  paraparesis  of  multiple  sclerosis  As  made  possible  by  demonstration  of 
the  purely  atrophic  optic  affection,  which  is  rarely  absent  in  this  disease,  of 
the  intention  tremor,  of  the  absence  of  the  abdominal  reflexes  in  the  early 
stages  of  multiple  sclerosis  (E.  Midler'). 

Prognosis. — The  forms  of  system  disease  that  have  been  discussed  here, 
permit  no  favorable  prognosis  in  the  majority  of  cases.  The  degenerative 
processes,  in  most  instances  spread  farther  and  farther  and,  together  with  the 
more  or  less  quickly  progressing  functional  limitations  and  the  complications 
caused  by  the  bladder  disturbances,  and  eventually  also  by  bed-sores,  lead, 
in  the  course  of  a  few  years,  to  death.     At  the  same  time  the  lessening  of  the 


318  DISEASES  OF  THE  SPINAL  CORD 

general  ability  to  resist,  due  to  the  constitutional  changes  which  are  the  basis 
of  the  disease,  diseases  of  the  blood,  chronic  intoxications  and  infections  have 
a  pernicious  influence.  Nevertheless,  the  disease  may  possibly  become 
stationary,  and  occasionally  with  an  improvement  in  the  general  condition,  a 
far  reaching  restitution,  leaving  only  slight  functional  defects,  may  take  place. 

The  therapy  must,  above  all,  take  into  consideration  the  general  con- 
dition of  the  patient  and  aim  at  getting  rid  of  toxic  substances,  at  the  im- 
provement of  the  conditions  of  nutrition,  and  in  anaemics,  so  far  as 
possible,  at  stimulating  the  formation  of  blood.  Therefore  the  dietetic  and 
medicinal  treatment  is  guided  preeminently  by  the  type  of  the  internal  dis- 
turbance. The  possiblity  of  a  syphilitic  disease  of  the  blood-vessels  must  not 
be  overlooked. 

In  respect  to  the  spinal  disturbances,  all  possible  care  and  rest  is  necessary 
for  the  purpose  of  avoiding  all  over-exertion  of  a  system  which  is  often  still 
capable  of  performing  its  functions  within  moderate  limits.  Therefore  all 
forced  movement  exercises  and  bath  procedures  are  contra-indicated.  On 
the  other  hand  solicitous  care  of  the  skin,  with  careful  furthering  of  diaphore- 
sis is  recommended;  the  emptying  of  the  bladder  is  to  be  supervised  and  care 
must  be  taken  to  keep  the  bowels  regular.  After  the  functional  disturbances 
have  become  stationary  for  a  long  time,  and  when  there  is  an  obvious  improve- 
ment in  the  general  strength  of  the  patient,  slowly  increasing  exercises  may 
gradually  be  begun.  Galvanization  of  the  back  along  the  spinal  column  may 
be  recommended.  In  the  grave  progressive  cases,  great  care,  a  position  in 
bed  calculated  to  avoid  bed-sores,  etc.,  may  help  in  sparing  the  patient,  who 
is  kept  in  bed  by  ataxia  and  paralysis,  painful  suffering. 

(i)  Bulbar-paralytic  Diseases 

BY 

FR.  JAMIN  (Erlangen) 
i.  Progressive  Bulbar  Paralysis 

Paralysis  Glosso-labio-pharyngea  Progressiva 

Progressive  bulbar  paralysis  belongs  to  the  systemic  diseases  of  the  motor 
nervous  system  and  is  closely  related  to  the  spinal  diseases,  which  are  charac- 
terized by  primary  destruction  of  the  peripheral  motor  neuron  and  the  clinical 
phenomena  of  paralysis  and  degenerative  muscular  wasting.  Even  in  spinal 
progressive  muscular  atrophy  frequently,  and  in  amyotrophic  lateral  sclerosis 
almost  regularly,  besides  the  gray  anterior  columns  of  the  spinal  cord,  the 
nuclei  of  the  motor  cerebral  nerves  which  are  of  equal  functional  importance, 
are  gradually  implicated  by  the  disease  process  in  the  form  of  progressive 
atrophy.  The  nuclei  of  the  nerves  of  the  eye  muscles  are  an  exception  to  this. 
In  rare  cases,  for  reasons  hitherto  unknown,  the  primary  disturbance  of  the 


PROGRESSIVE  BULBAR-PARALYSIS  319 

motor  system  is  limited  from  the  first  to  these  bulbar  motor  nuclei  of  the 
medulla  oblongata  and  the  region  of  the  pons,  and  then  there  develops  the 
well  characterized  symptom  complex  of  a  progressive  paralysis  and  muscular 
atrophy  in  the  areas  of  the  musculature  of  the  tongue,  face,  palate,  pharynx 
and  larynx,  the  picture  of  progressive  bulbar  paralysis. 

With  the  exception  of  the  rather  rare  atypical  cases  in  childhood,  the 
disease  is  found  as  a  rule  at  a  somewhat  advanced  age,  generally  near  the 
fiftieth  year,  scarcely  before  the  thirty-fifth  or  fortieth.  Familial  appearance 
has  been  very  seldom  reported — as  a  rule  the  cases  are  isolated.  Yet  the 
assumption  of  congenital  weakness  of  the  bulbar  nuclear  centers  as  a  foun- 
dation for  the  disease  is  not  unjustifiable,  on  the  analogy  of  the  other  related 
systemic  motor  diseases  and  in  the  absence  of  definitely  proven  exogenous 
causes.  How  far  one  is  justified  in  regarding  colds,  traumatisms,  emotional 
disturbances  as  causal  factors,  a  critical  analysis  of  each  individual  case  must 
decide.  It  is  not  improbable  that  the  over-exertion  of  the  injured  muscular 
regions,  especially  of  the  muscular  apparatus  of  the  buccal  cavity  in  certain 
occupations  (blowing  musical  instruments,  glass  blowing,  etc.),  has  a  pecu- 
liarly injurious  influence,  if  a  diminished  power  of  resistance  is  present  at  the 
same  time.  Possibly  the  diminished  resistance  to  the  demands  made  upon 
the  economy  by  daily  life,  which  is  a  sequela  of  syphilis  and  other  infections 
or  intoxications,  may  also  be  limited  to  these  regions. 

Course  of  the  Disease  and  Symptomatology. — The  phenomena  of  the 
disease  develop  very  slowly  and  insidiously,  as  in  progressive  muscular 
atrophy.  Step  by  step  in  the  comparatively  narrow  field  of  action,  one  section 
after  another  is  deprived  of  its  function,  in  irresistable  succession.  Since  the 
muscles  here  concerned  represent,  in  many  respects,  the  guardians  and  the 
assisting  preparatory  forces  for  the  orifices  into  the  gastro-intestinal  canal  and 
the  respiratory  passage,  their  functional  disturbance  soon  causes  a  disturbance 
of  the  vital  processes,  nutrition  and  respiration.  Therefore  in  every  bulbar 
motor  disturbance,  the  duration  of  the  disease  in  comparison  with  that  of  the 
other  systemic  diseases,  which  affect  only  the  locomotor  capacity  of  the 
patient,  is  limited.  Thus  progressive  bulbar  paralysis,  in  spite  of  its  usually 
regular,  slow  development,  which  rarely  proceeds  by  greater  strides,  following 
one  another  more  quickly,  ends  in  death  after  a  few  (two  to  five)  years  by 
disturbances  in  nutrition,  or  by  diseases  of  the  respiratory  organs. 

The  first  signs  of  the  disease  become  noticeable  to  the  patient  himself 
and  to  those  surrounding  him  through  difficulty  in  speech.  The  extraordi- 
narily fine  degrees  of  movements  of  the  tongue,  that  are  essential  in  manifold 
combinations  for  the  production  of  sound,  are  already  influenced  at  a  time 
when  the  disjunction  of  separate  contractile  elements  in  the  midst  of  the 
mighty  muscular  mass  of  the  tongue,  could  not  have  altered  its  simple 
general  movements  and  its  general  noticeable  volume.  In  progressive 
bulbar  paralysis,  likewise,  muscular  wasting  and  paralysis  go  hand  in  hand; 


32o  DISEASES  OF  THE  SPINAL  CORD} 

but  the  functional  abilities,  which  have  been  acquired  with  exceptional 
difficulty,  and  which  are  essential,  especially  for  the  complicated  mechanism  of 
speech,  so  influence  bulbar  paralyses,  that  the  functional  disturbance  must  be 
noticeable  quite  early,  even  in  the  slightest  injury  to  the  muscular  pliability  and 
action,  and  before  the  myoatrophic  loss  is  visible.  The  composition  of  the 
sentences  and  the  placing  of  words  and  sounds  are  altogether  undisturbed, 
only  pronunciation  becomes  difficult,  confused,  unclear,  and  thick;  it  is  a 
matter  of  a  pure  articular  or  dysarthric  disturbance  of  speech.  At  first,  the 
utterance  of  the  lingual  sounds  suffers  (D,  T,  L,  R,  N,  S,  Sh,  I)  in  which  the 
participation  of  the  soft  palate  soon  makes  itself  felt  by  a  nasal  accessory 
sound  and  hindrance  in  the  formation  of  the  sounds  (C,  K,  Ch)  produced 
in  the  back  part  of  the  buccal  cavity.  Afterwards,  the  labial  sounds  are 
also  more  and  more  affected  (B,  P,  F,  M,  W,  O,  U,  E).  The  weakness  of 
the  muscles  of  the  larynx  which  is  added  late,  causes  hoarseness,  a  loss  of  tone 
in  the  voice,  monotony  in  a  deep  range,  finally  aphonia,  so  that  the  patient 
can  make  himself  understood  only  with  difficulty  because  of  an  inarticulate 
thickness  of  speech  and  stammering. 

Soon  after  the  appearance  of  the  first  speech  disturbances,  the  first  diffi- 
culties in  swallowing  become  apparent.  The  awkward  tongue  is  unable  to 
direct  the  food  which  is  in  the  mouth  correctly  down  into  the  throat;  the 
paralysis  of  the  soft  palate  frequently  causes  a  regurgitation  of  fluid  food 
from  the  nasal  cavity;  solid  food,  because  of  the  insufficiently  functioning 
epiglottis  and  of  imperfect  occlusion  of  the  glottis,  reaches  the  larynx  and 
the  trachea;  the  musculature  of  the  throat  loses  its  ability  to  force  the  bits 
of  food  into  the  oesophagus. 

If  the  patients  are  examined  at  this  time  of  obvious  functional  disturb- 
ances, the  bulbar  paralyses  and  atrophies  are  usually  found  present. 

The  facial  muscles  are  wasted  in  the  lower  half  of  the  face.  Especially 
the  lips  seem  thin,  wrinkled.  They  cannot  be  closed  satisfactorily,  nor  can 
the  mouth  be  pursed;  whistling  is  impossible.  At  the  same  time,  the  muscles 
of  the  forehead  and  the  eyelids,  as  well  as  of  the  eyes,  as  a  rule  remain  intact 
and  functionate.  The  tongue  can  be  moved  only  with  difficulty  and  in- 
sufficiently. It  lies  limp  in  the  buccal  cavity,  its  surface  is  wrinkled,  crossed 
by  deep  furrows,  has  thin  edges  (Fig.  72)  and  displays  active  fibrillary 
tremors,  which  may  also  appear  in  the  lips.  The  uvula  hangs  low,  and  is  but 
little  raised  in  phonation,  if  at  all.  The  immobility  of  the  floor  of  the  mouth 
and  of  the  base  of  the  tongue  is  completed  by  the  paralysis  and  atrophy  of  the 
muscles  inserted  in  the  hyoid  bone  from  the  innervation  region  of  the  tri- 
facial, the  facial,  and  the  hypoglossal  nerves.  A  laryngoscopic  examination 
discloses  the  imperfect  occlusion  of  the  glottis  by  means  of  a  phona- 
tion test.  Active  swallowing  movements  and  the  raising  of  the  larynx  con- 
nected with  them  are  lost.  A  paralysis  of  the  muscles  of  chewing  also 
appears  in  many  cases  with  atrophy  of  the  temporals  and  the  masseters 


PROGRESSIVE  BULBAR  PARALYSIS 


321 


and  an  inability  to  shut  the  jaws  properly  and  to  make  extensive  lateral 
movements  of  the  lower  jaw. 

In  the  affected  muscles,  single  fibres  and  bundles  of  fibres  are  retained, 
and  remain  functional  for  some  time,  so  that  for  a  long  time  the  change  in 
electrical  excitability  can  not  be  demonstrated  at  all,  or  only  with  difficulty. 
But  in  the  atrophic  tqngue,  in  the  thin  pliable  lips,  a  partial  reaction  of 
degeneration  is  frequently  found,  and  the 
sluggish  character  of  the  contraction  in 
direct  galvanic  stimulation  is  unmis- 
takable. 

The  reflex  excitability  of  the  atro- 
phied muscles  is,  as  a  rule,  considerably 
weakened,  or  even  lost.  In  several 
cases,  however,  analogous  to  the  phe- 
nomena of  amyotrophic  lateral  sclero- 
sis, obviously  with  simultaneous  lesion 
of  the  proper  central  (pyramidal)  tracts, 
an  exaggeration  of  the  reflexes  in  the 
facial  region  and  especially  of  the 
masseter  reflexes  could  be  established. 
On  the  other  hand,  the  palatal  and 
throat  reflexes  are  usually  altogether 
lost.  In  "swallowing  the  wrong  way" 
which  is  of  frequent  occurrence,  re- 
flexly  excited  coughing  appears,  but 
since  an  expiratory  increase  of  pressure 
in  the  air  passages  is  impossible  because 
of  the  insufficient  closing  of  the  glottis, 
the  hoarse  coughing-up  lacks  effective 
force.     Therefore   foreign  bodies,  food 

and  bronchial  secretions  can  no  longer  be  ejected,  and  there  results  the 
very  dangerous  deglutition  pneumonias,  serious  bronchitis,  broncho-pneu- 
monic processes  and  gangrenous  local  lesions  in  the  lungs. 

The  sensibility  even  in  the  area  of  the  trigeminus  is  never  disturbed  in 
any  respect,  nor  the  function  of  the  sense  organs  or  of  the  sense  of  taste. 
Slight  vaso-motor  disturbances,  rush  of  blood  to  the  head  with  a  feeling  of 
heat,  and  hyperemia  occur.  Observations  of  a  considerable  increase  in  the 
pulse  rate  seem  to  indicate  that  the  vagus  is  affected. 

Paralysis  and  muscular  wasting  appear  first  in  the  tongue,  then  in  the  lips 
and  in  the  muscles  of  the  lower  part  of  the  face;  finally  in  the  soft  palate,  the 
floor  of  the  oral  cavity,  pharynx,  larynx,  and  the  muscles  of  chewing.  Hence 
the  paralysis  is  restricted  to  the  hypoglossus,  facialis  in  its  lower  division, 
motor   trigeminus   and  vagus   accessorius.     Only  rarely  do  the  motor  dis- 


FiG.  72. — Atrophy  of  the  tongue  in  in- 
fantile progressive  bulbar  paralysis.  Flac- 
cid features.  Because  of  the  co-affection  of 
the  upper  region  of  the  facial  the  lids  are 
not  perfectly  closed.  (After  Schoenborn  and 
Krieger.) 


322 


DISEASES  OF  THE  SPINAL  CORD 


turbances  spread  to  the  muscles  of  the  neck,  the  shoulder  girdle  and 
the  extremities.  The  upper  facialis  area  is  only  exceptionally  affected 
(Oppenheim) . 

In  the  later  stages  of  the  disease,  a  -proinse  flow  of  saliva,  which  is  very 
annoying  to  the  patient,  is  nearly  always  observed.  In  part,  this  may  be 
actually  due  to  a  secretory  disturbance  with  increased  production  of  saliva. 
But,  on  the  other  hand,  salivation,  the  continuous  out-flow  of  saliva  from  the 
corners  of  the  half -open  mouth,  is  certainly  evoked  indirectly  also  by  the  dis. 


Fig.  73. — Progressive  bulbar  paralysis.  "Transverse  laughing"  and  impossibility  of  closing 
the  lips  because  of  atrophy  of  the  facial  muscles.  The  emaciated  face  shows  folds  because  of  the 
"  skin  having  become  too  wide."     (After  Schoenborn  and  Krieger.) 


turbances  in  movement,  so  that  the  saliva  produced   can  no  longer  be 
swallowed. 

The  completely  developed  picture  of  progressive  bulbar  paralysis  is  very 
characteristic  (Fig.  73).  The  face  is  rigid,  thin,  and  wrinkled,  only  the 
movements  of  the  forehead  and  eyes  give  expression  to  the  intellectual  life. 
The  mouth  is  somewhat  open,  the  lower  lip  hangs  down  and  permits  the 
saliva  to  escape,  the  corners  of  the  mouth  droop.  In  attempting  facial 
expression,  in  crying  or  laughing,  the  mouth  is  drawn  slightly  outwards, 
which  gives  the  face  a  distorted,  suffering  look.  The  inanition  produced  by 
the  disturbance  in  swallowing  still  further  increases  the  prominence  of  the 
morbid  emaciation,  since  every  particle  of  the  cushion  of  fat  is  absorbed. 


PROGRESSIVE  BULBAR  PARALYSIS  323 

Pathological  Anatomy. — To  the  selective  injury  of  the  function  and 
nutrition  of  the  muscles  from  a  part  of  the  region  of  the  motor  cerebral  nerves, 
corresponds  in  the  anatomic  picture  of  the  paralysis  glosso-labio-pharyngea, 
an  atrophy  of  the  nuclei  of  the  cerebral  nerves  concerned.  In  the  nucleus  of 
the  hypoglossus,  wasting  and  atrophy  of  the  ganglion  cells  are  most  clearly 
defined,  but  they  are  found  also  in  the  nuclei  of  the  facialis,  the  motor 
trigeminus,  and  the  motor  parts  of  the  glosso-pharyngeus  and  vagus-acces- 
sorius.  Instead  of  the  wasted  ganglion  cells,  and  the  network  of  nerve  fibres, 
which  has  been  for  the  most  part  destroyed  in  the  degenerated  nuclei,  there 
appear  proliferation  of  the  glia  fibres  and  thickening  of  the  vessels,  but  with- 
out any  decided  inflammatory  changes.  The  motor  roots  of  the  nuclei  that 
have  wasted,  are  degenerated,  likewise  the  appertaining  peripheral  motor 
nerve  fibres  down  into  the  muscles,  which  themselves  show  advanced  atrophy 
according  to  the  manner  of  the  spinal  muscular  atrophy.  In  some  cases, 
besides  this,  a  participation  of  the  pyramidal  tracts  also,  just  as  in  amyo- 
trophic lateral  sclerosis,  has  been  found  to  be  present.  The  close  relations 
of  progressive  bulbar  paralysis  to  this  and  to  spinal  muscular  atrophy  have 
been  mentioned  at  the  beginning.  They  are  of  one  type,  as  primary  selective 
atrophic  processes  of  the  motor  nervous  system,  the  clinical  appearance  of 
which  depends  only  upon  whether  the  localization  of  the  injury  is  to  be  found 
in  the  central  tracts,  the  bulbar  or  the  spinal  nuclei. 

The  diagnosis  of  progressive  bulbar  paralysis  offers  no  great  difficulties, 
if  the  course  is  considered  which  usually  advances  very  gradually  in  typical 
succession  from  the  musculature  of  the  tongue  to  that  of  the  face  and 
pharynx,  as  well  as  the  nature  of  the  disease  as  a  purely  degenerative  atrophic 
paralysis  within  the  boundaries  defined.  Differentiation  from  the  rest  of  the 
motor  systemic  diseases  is  founded  upon  the  condition  of  the  musculature  of 
the  body  and  the  extremities,  possibly  upon  the  presence  of  spasms.  The 
similarly  localized  paralyses  in  acute  bulbar  diseases  show  a  different  course, 
and  scarcely  ever  remain  so  free  from  participation  of  the  tracts  for  move- 
ment in  the  extremities,  and  for  sensory  conduction-tracts  as  they  are  so 
closely  packed  together  in  the  medulla  oblongata.  In  these,  as  in  bulbar 
gliosis,  multiple  sclerosis,  and  tumors,  the  lesion  of  the  cerebral  nerves, 
especially,  is  more  extended,  not  so  peculiarly  circumscribed,  attacking  also 
the  muscles  of  the  eye  and  the  organs  of  sense.  Pseudo-bulbar  paralysis  (cf. 
p.  333)  is  distinguished  from  the  genuine  paralysis  by  the  swifter,  frequently 
quite  sudden  or  jerky  progress  of  the  disease  picture,  by  many  phenomena 
which  suggest  a  diffuse  participation  of  the  brain  (psychic  disturbances, 
paresis  of  the  extremities)  and  by  the  preservation  of  a  far-reaching  reflex 
ability  in  the  paralyzed,  but  not  limp  atrophic  bulbar  muscles. 

The  prognosis  of  atrophic  bulbar  paralysis  is  absolutely  unfavorable. 
The  affection  is  incurable  in  its  phenomena  of  nervous  functional  loss,  and 
because  of  the  disturbances  in  swallowing,  and  the  insufficient  protection 


324  DISEASES  OF  THE  SPINAL  CORD 

of  the  air  passages,  these,  in  turn,  soon  cause,  as  has  been  mentioned 
above,  complications  in  the  lungs  and  bronchial  tubes;  malnutrition  is 
another  important  factor.  Then,  too,  the  paralysis  of  the  larynx  may  lead 
directly  to  dangerous  attacks  of  choking,  and,  under  certain  conditions,  as 
result  of  a  disturbance  in  the  central  regulation  of  the  heart's  activity,  asso- 
ciated with  exhaustion,  sudden  paralysis  of  the  heart  may  appear. 

Nevertheless,  the  pitiful  condition  of  the  patient,  who  with  an  entirely 
clear  intellect,  suffers  extremely,  demands  great  care  and  treatment.  Beside 
the  general  strengthening  by  means  of  baths,  nourishing  foods  and  various 
restorative  procedures  at  the  beginning  of  the  disease,  an  attempt  should  be 
made  to  strengthen  the  resistance  of  the  muscles  retained,  in  the  slightly 
disturbed  parts,  by  galvanization  of  the  nuchal  region,  also  by  tonics  (strych- 
nine, nitrate  of  silver).  As  long  as  the  cause  is  not  entirely  clear,  treatment 
with  iodide  of  potassium  should  not  be  omitted.  At  the  beginning  of  the 
difficulties  in  swallowing,  nutrition  finds  serious  tasks.  Now  it  is  a  question 
of  reducing,  so  far  as  possible,  the  trouble  of  swallowing  by  a  fluid  diet,  or 
one  of  thin  porridge,  with  corresponding  assistance;  the  artificial  food 
preparations  are  here  very  useful  and  are  still  more  necessary  when,  because 
of  too  great  danger  of  choking,  the  food  must  be  taken  by  means  of  a  tube 
either  through  the  nose  or  throat — the  nasal  tube  being  occasionally  more 
easily  inserted.  The  slow  contractions  of  the  atrophic  pharyngeal  muscula- 
ture, which  can  be  evoked  by  galvanization  in  the  neck  further  the  swallowing 
act,  and  have,  at  least,  the  good  suggestive  influence  in  that  the  patient  can 
then  feel  the  swallowing  movements  which  he  can  no  longer  actively  perform. 
The  excessive  flow  of  saliva  can  be  combated  by  atropin.  Even  though  the 
affection  in  itself  causes  no  pain,  still,  in  the  last  stages,  the  restless  condition 
of  the  patient,  who  is  tortured  by  difficulties  in  swallowing,  by  the  violent 
and  ineffectual  cough,  by  difficulty  in  breathing,  and  a  feeling  of  oppression 
in  the  heart,  demands  the  administration  of  narcotics. 

2.  Acute  (Apoplectic)  Bulbar  Paralysis 

Phenomena  of  paralysis  in  the  areas  of  the  motor  cerebral  nerves  after 
the  manner  of  bulbar  paralysis  may  develop  in  acute  form  also,  if  a  sudden 
injury  strikes  the  motor  nuclei  situated  in  the  regions  of  the  pons,  and  in 
the  medulla,  the  roots  or  the  supranuclear  tracts  of  the  cerebral  nerves. 
These  acute  bulbar  paralyses  appear  but  rarely  in  so  symmetrical  an  arrange- 
ment as  the  movement  disturbances  in  the  slowly  advancing  atrophic  bulbar 
paralysis,  and  only  in  the  rare  cases,  in  which  a  disease  corresponding  to 
acute  poliomyelitis  anterior  attacks  the  bulbar  nuclear  fields  alone,  or  as 
bulbar  infantile  paralysis,  together  with  the  inflammation  of  the  gray 
anterior  columns  of  the  spinal  cord,  do  they  remain  wholly  restricted  to  the 
functional  disturbances  of  the  motor  cranial  nerves.     In  the  more  frequent 


ACUTE  BULBAR  PARALYSIS  325 

lesions  of  the  nuclei  of  the  motor  cerebral  nerves  due  to  hemorrhages, 
thrombotic  or  embolic  softening,  traumatic  degeneration  and  acute  inflam- 
mation in  the  medulla  oblongata  and  the  pons,  the  neighboring  sensory 
centers  and  conduction  tracts,  the  motor  tracts  for  the  extremities  and  the 
rest  of  the  central  apparatus  of  the  medulla  oblongata  do  not  escape. 
According  to  the  location  and  the  extent  of  the  central  lesion,  there  are 
formed,  in  manifold  combinations,  complicated  disease  pictures,  which  are 
especially  characterized  by  the  appearance  of  alternating  and  crossed 
paralyses  and  alternating  phenomena  of  sensory  functional  loss. 

Etiology  and  Pathological  Anatomy. — Acute  bulbar  paralysis  is 
most  frequently  caused  by  diseases  of  the  blood-vessels.  Arterio-sclerotic 
changes  in  the  basilar  and  in  the  vertebral  arteries,  as  well  as  in  their  end 
twigs,  lead,  through  narrowing  of  the  lumen  of  the  arteries  to  insufficient 
blood  supply  of  the  bulbus,  and  sometimes  even  by  complete  thrombotic 
occlusion  to  ischaemic  necrosis  in  the  neighborhood.  The  branches  of  these 
arteries,  especially  the  arteria  cerebelli  inferior  posterior,  which  springs  from 
the  vertebralis,  can  to  this  extent  be  regarded  as  end  arteries  in  that  usually, 
when  there  is  sudden  closure  and  difficulty  in  the  circulation,  as  a  result  of 
general  atheromatosis  of  the  cerebral  arteries,  sufficient  collateral  circulation 
fails  to  develop.  The  consequence  of  such  occlusion  of  the  blood-vessels, 
which  in  rarer  cases  can  also  be  produced  by  an  embolus  from  the  heart, 
especially  in  the  left  vertebral  artery,  is  the  local  death  of  tissue,  with  conse- 
quent softening  and  formation  of  cysts.  Similar  changes  appear,  also,  in 
consequence  of  syphilitic  endarteritis.  In  rarer  cases,  arteriosclerotic 
changes  and  the  formation  of  miliary  aneurisms,  especially  under  the 
influence  of  factors  that  increase  the  pressure  of  the  blood,  cause  larger 
focal,  or  small  multiple  hemorrhages  with  destruction  of  tissue  in  the 
substance  of  the  pons  and  the  medulla.  Larger  aneurismal  dilatations 
of  the  basilar  can,  just  as  other  narrowing  processes  do,  through  compression 
of  the  medulla,  lead  to  bulbar  disturbances;  in  their  most  destructive  form, 
they  are  seen  in  fractures  and  dislocations  of  the  upper  two  cervical  vertebrae. 
Severe  traumatisms  upon  the  back  of  the  head,  can  also  give  rise  to  hemor- 
rhages in  the  region  of  the  bulbar  centers. 

Besides  these  softenings  or  hemorrhagic  disturbances  caused  by  changes 
or  injuries  in  the  arterial  system,  inflammatory  encephalitic  bulbar  diseases 
are  observed,  which,  especially  in  youth,  appear  in  connection  with  infectious 
diseases,  or  even  in  the  wake  of  toxic  processes.  Perivascular  infiltrations, 
small  hemorrhages,  degenerations  of  the  nerve  fibres  and  the  ganglion  cells, 
granular  cells,  proliferation  of  the  vessels  then  disclose  on  microscopic 
examination  a  more  diffuse  injury  to  the  nervous  system.  Lighter  dis- 
turbances of  this  kind  may  be  recovered  from  before  a  more  deeply  rooted 
injury  of  the  nervous  substance  has  occurred.  Such  cases  exhibit  but 
temporarily  the  symptomatic  picture  of  acute  bulbar  paralysis;  in  a  similar 


326  DISEASES  OF  THE  SPINAL  CORD 

way,  but  without  participation  of  the  long  conducting  tracts,  it  may  be 
simulated  also  by  a  multiple  neuritis  of  the  bulbar  nerves.  We  must  like- 
wise mention  here,  as  a  very  rare  occurrence,  abscess  of  the  medulla  oblon- 
gata, which  is  usually  metastatic. 

Symptomatology. — The  phenomena  of  acute  bulbar  paralysis  appear  in 
those  cases  in  which  they  are  evoked  by  hemorrhages  or  thrombotic  soften- 
ing, stroke-like,  after  the  manner  of  a  stroke  of  apoplexy.  As  a  rule,  slighter 
subjective  complaints  like  headaches,  especially  in  the  back  of  the  head, 
and  attacks  of  dizziness  precede  for  some  time,  or,  perhaps,  for  a  few  days 
only,  as  signs  of  the  disease  of  the  blood-vessels,  which  frequently  accom- 
panies chronic  nephritis,  then  the  patient  suddenly  falls  down  with  a  violent 
attack  of  vertigo,  associated  with  headache.  Consciousness  is  often  retained, 
but  may  be  lost.  Vomiting,  tinnitus  aurium,  clonic  twitchings,  in  rare  cases 
(especially  in  hemorrhages  of  the  pons),  a  single  or  even  repeatedly  occur- 
ring epileptoid  convulsive  seizures  accompany  the  stormy  outbreak  of  the 
phenomena.  As  a  rule,  the  symptoms  of  labio-glosso-pharyngeal  paralysis 
may  then  be  immediately  recognized.  Occasionally,  a  few  days  may  elapse 
before  the  picture  of  the  disease  is  fully  developed.  Although  the  paralysis 
of  the  cerebral  nerves  and  also  of  the  extremities  is  at  first  unilateral  it  may, 
as  the  occlusion  of  the  vessels  progresses,  seize  upon  the  other  half  of  the 
body,  and  an  extension  of  the  lesion  upwards  or  downwards  may  be  recog- 
nized by  the  fact,  that  ever  more  and  more  bulbar  functions  are  being 
attacked.  In  cases  of  inflammatory  bulbar  affections,  the  development 
of  the  disease  may  occur  also  in  a  subacute  manner,  and  extend  over  a  num- 
ber of  days  or  weeks. 

The  articulatory  dysarthria  disturbances  in  speech,  which  appear  with 
retained  sensorium,  or  when  consciousness  returns  immediately  after  the 
apoplectic  attack,  as  a  result  of  the  paralysis  of  the  muscles  of  the  tongue  and 
larynx,  are  specially  characteristic.  Speech  becomes  thick,  difficult  to 
understand,  hoarse  and  aphonic.  To  this  is  added  the  early  appearing  deglu- 
tition paralysis,  combined  with  paralysis  of  the  soft  palate,  and  the  loss  of  the 
pharyngeal  and  palatal  reflexes.  The  patients  can  not  swallow  either  solid 
or  liquid  food.  The  latter  regurgitates  through  the  nose;  frequently,  also,  the 
food  goes  into  the  windpipe,  causing  a  troublesome  cough  which,  because 
of  the  inefficient  closure  of  the  glottis,  does  not  lead  to  sufficient  expectora- 
tion. The  paralysis  of  the  face  is  still  more  striking.  This  frequently 
attacks  all  the  branches  of  the  facialis,  causing  a  relaxation  of  the  features, 
insufficient  closure  of  the  lids  and  impossibility  of  active  or  reflex  movements 
of  the  facial  muscles,  occurring  usually  on  one  side  but  occasionally  on  both. 
This  facial  paralysis  is  often  followed  by  an  abducens  paralysis  on  the 
same  side,  or  the  inability  to  direct  vision  to  the  side  of  the  paralysis 
(cf .  below) .  The  muscles  of  chewing  are  not  rarely  attacked  by  the  paralysis ; 
in  other  cases,  especially  at  the  beginning  of  the  disturbance,  because  of 


ACUTE  BULBAR  PARALYSIS 


327 


central  irritation  due  to  a  focus  in  close  proximity  to  one  of  the  motor  nuclei 
of  the  trigeminus,  trismus  is  observed. 

According  to  the  location  and  extent  of  the  central  lesion,  the  motor 
disturbance  in  the  cerebral  nerves,  and,  when  the  pyramidal  tracts  participate, 
in  the  extremities  also,  may  assume  various  forms  in  various  combinations 
(cf.  Figs.  74  and  75). 

If  the  disturbance  extends  cranially  up  to  the  region  of  the  cerebral 
peduncles  and  the  area  of  the  corpora  quadrigemina,  the  central  tracts  for  the 
extremities  and  for  the  seventh,  tenth  and  twelfth  cerebral  nerves,  in  cases 
of  one-sided  focal  disease,  are  affected  above  their  crossing,  together  with 
the  nuclei  of  the  oculomotor,  and  there  appears  a  hemiplegia  alternans 
superior  (Weber) :  a  paralysis  of  the  oculomotor  on  the  side  of  the  lesion,  and 
a  supranuclear  paralysis  of  the  bulbar  nerves  and  the  extremities  on  the 
opposite  side  (Fig.  75,  77). 


Corpora  quadrigemina 


Nucleus  offortinu 
column 


Pons 


Fig.  74. — Schematic  view  of  the  location  of  the  nuclei  III  to  XII.  Motor  nuclei  dotted, 
sensory  deep  black.  XIX  means  X  and  IX  (common  nucleus  of  Nr.  IX  and  X).  (After 
Villiger) . 

Hemiplegia  alternans  inferior  (Gubler),  in  which  the  nucleus  of  the  facialis, 
or  its  already  crossed  supranuclear  tract,  is  drawn  into  the  focal  injury, 
whereas  the  pyramidal  tract  is  destroyed  above  the  crossing  in  the  pons,  is 
more  frequently  found,  so  that  a  paralysis  of  the  face,  usually  flaccid,  is 
combined  with  a  spastic  paralysis  in  the  extremities  on  the  opposite  side.  At 
the  same  time  there  is  often  a  paralysis  of  the  abducens  on  the  side  of  the 
lesion,  or,  if  the  nucleus  of  the  abducens  and  its  surroundings,  with  the 
connections  with  the  nuclei  of  the  muscles  of  the  eye  are  injured,  there  is  an 
associated  ocular  paralysis;  the  ability  of  both  bulbi  to  turn  to  the  side  of 
the  lesion  is  lost  (Fig.  75,  III  and  IV). 

If  the  focal  disease  is  confined  to  the  deeper  parts  of  the  medulla  oblongata, 
the  unilateral  lesion  of  the  nuclei  of  the  vagus-accessorius  and  of  the  hypo- 
glossus  or  its  motor  roots,  may  strike  the  pyramidal  tracts  above  the  crossing, 


328  DISEASES  OF  THE  SPINAL  CORD 

and  then  there  occurs  a  paralysis  of  the  tongue  and  the  muscles  of  swallowing, 
as  well  as  of  the  soft  palate  on  the  side  of  the  lesion,  which,  moreover,  leads 
from  the  onset  to  severe  disturbances  in  deglutition — and  paralysis  of  the 
extremities  on  the  opposite  side  (hemiplegia  alternans  infima)  (Fig.  75,  V). 

Should  the  central  injury  finally  strike  the  pyramidal  crossing  also,  it  may 
happen  that  the  central  tract  for  the  upper  extremity  is  interrupted  above  the 
crossing,  while  that  for  the  lower  extremity  may  be  affected  below  the  cross- 
ing; then  the  arm  on  the  side  of  the  lesion  and  the  leg  on  the  opposite  side, 
will  be  paralyzed,  i.  e.,  exhibit  a  spastic  paresis  {hemiplegia  cruciata)  or 
paralysis  of  both  legs  may  appear  or,  more  rarely,  of  both  arms. 

Since,  owing  to  the  small  size  of  the  pons  and  the  medulla,  the  softening 
and  inflammatory  foci  frequently  pass  beyond  the  middle  line,  and  as  the 
boundaries  of  the  foci  are,  as  a  rule,  irregular,  there  occur  also  bilateral 
disturbances,  paralysis  of  all  the  extremities,  combined  with  unilateral 
bulbar  nuclear  paralysis  in  the  facialis  or  of  the  oral  or  pharyngeal  cavities, 
or  even  bilateral  glosso-labio-pharyngeal  paralysis,  connected  with  unilateral 
paresis  of  the  extremities. 

The  motor  tracts  for  the  extremities  are,  in  bulbar  focal  lesions,  always 
affected  above  the  spinal  motor  nuclei;  the  paralysis  of  the  extremities,  there- 
fore, corresponds  in  its  details  to  cerebral  hemiplegia;  if  the  disease  con- 
tinues long  enough,  there  develops  a  spastic  paresis  of  the  extremities.  Not 
infrequently,  however,  the  tendon  reflexes  are  lost,  especially  in  the  upper 
extremities,  probably  as  a  result  of  synchronous  lesion  of  the  spino-cerebellar 
tracts;  the  Babinski  reflex  also  does  not  appear  regularly  in  bulbar  paralysis. 

The  paralysis  in  the  areas  of  the  cranial  nerves  is  in  most  cases  of  acute 
bulbar  paralysis,  throughout,  or  in  the  greater  part  of  the  paralyzed  region, 
degenerative,  flaccid,  corresponding  to  the  lesion  of  the  peripheral  motor 
neuron  in  the  nuclear  area,  or  in  the  motor  roots.  This  may  be  demonstrated 
in  the  individual  case  by  the  absence  of  reflex  excitability  in  the  musculature  of 
the  face,  tongue,  and  throat.  If  the  paralysis  persists  for  several  days,  the 
presence  of  a  quickly  progressing  atrophy  in  the  paralyzed  facial  muscles  and 
in  the  tongue,  as  well  as  fibrillary  Switchings  and  electric  reactions  of  degenera- 
tion may  be  seen  to  develop  in  many  cases.  The  latter  may  occasionally  be 
evoked  in  the  musculature  of  the  pharynx  also. 

Next  to  the  motor  disturbances  which  are  mostly  of  an  alternating 
character,  the  condition  of  sensation  in  acute  bulbar  paralysis  deserves 
attention.  Here,  also  it  is  to  be  remembered  that  the  conduction  of  the 
sensibility  of  the  trunk  and  the  extremities,  undergoes,  for  the  most  part,  a 
decussation  in  the  higher  parts  of  the  medulla  oblongata,  so  that  with  a  focus 
that  appears  on  one  side  in  the  pons,  the  sensory  tracts  of  the  trunk  and  the 
extremities  may  be  affected  on  the  opposite  side,  in  addition  to  the  sensory 
roots  and  centers  of  the  trigeminus  on  the  same  side  of  the  lesion;  this  would 
result  in  a  hemianesthesia  cruciata,  with  paralysis  of  sensation  in  the  trunk 


ACUTE  BULBAR  PARALYSIS 


329 


and  the  extremities  on  the  opposite  side  and  loss  of  sensation  in  the  face 
(especially  in  the  second  and  third  branch  of  the  trigeminus)  and  in  the  oral 
and  pharyngeal  cavities  on  the  side  of  the  focal  injury.  In  the  deeper  divi- 
sions of  the  medulla  oblongata  (olivary  region)  relations  are  complicated  by 


Wind. 


Pyramidal  -\-- Anterior  pyramidal  trad 

crossing 

Spiral  mortar 

roots  to  right 

arm. 

Fig.  75. — Scheme  of  explanation  of  hemiplegia  alternans  (for  the  seventh  and  twelfth  nerves). 
Red:  pyramidal  tract;  N.VII  s,  left  facial  nerve;  N.XII  d,  right  hypoglossal  nerve.  I,  focus  in 
the  right  inner  capsule  left  half  of  the  body,  the  left  seventh  and  twelfth  nerves  affected;  II,  focus 
in  the  right  peduncle,  the  same  result;  III,  focus  in  the  right  half  of  the  pons,  left  part  of  the  body 
and  the  left  twelfth  nerve,  the  right  seventh  nerve  (supranuclearly)  affected;  IV,  the  same  caudad, 
left  part  of  the  body,  left  half  of  the  tongue  and  the  right  seventh  nerve  affected^(nuclearly  resp. 
radicularly) ;  V,  a  focus  in  the  medulla  paralyzes  the  left  half  of  the  body  and  the  right  half  of  the 
tongue  (nuclearly  or  radicularly) .     {After Liepman.) 


the  fact  that  in  near  vicinity  of  each  other,  three  sensory  systems  of  various 
importance  are  situated:  1.  In  the  medial  division,  near  the  raphe  (inter- 
olivary  layer)  the  kinesthetic  tracts  (sensation  of  position,  muscle  sense, 
pressure  sense — deep  sensibility),  which  are  crossed  here,  by  the  aid  of  the 


33©  DISEASES  OF  THE  SPINAL  CORD 

fibrae  arcutse  internse.  A  focus  situated  higher  up  will  therefore  cause  a 
disturbance  of  the  deep  sensibility  on  the  opposite  side,  a  lower  focus  may  be 
followed  by  a  destruction  of  the  deep  sensibility  on  the  side  of  the  lesion. 
2.  A  lesion  in  the  lateral  division  (tractus  spinotectales  and  spinothalamicae) , 
the  tracts  for  the  sensations  of  pain,  of  cold,  of  heat,  which  have  crossed 
already  in  the  spinal  cord,  brings  with  it  a  dissociation  disturbance  of  the 
sensations  of  pain  and  temperature  on  the  side  of  the  body  opposite  to  the 
focal  injury  (trunk  and  extremities).  3.  The  spinal  root  of  the  trigeminus 
with  the  substantia  gelatinosa,  which  is  the  medium  for  conduction  of  sensi- 
bility for  the  face,  especially  in  the  region  of  the  first  branch  of  the  trigeminus, 
in  all  its  qualities.  Since  the  fibres  of  the  trigeminus,  which  serve  for 
pain  and  temperature  sensation,  are  crossed  already  in  the  lower  sections 
of  the  medulla  oblongata,  a  focal  injury  here  may,  according  to  its  extent, 
cause  a  disturbance  in  sensibility  of  the  trigeminus  in  all  its  qualities 
on  the  side  of  the  lesion,  or,  it  may  produce  an  analgesia  and  therm- 
anaesthesia  on  the  opposite  side  of  the  face  (especially  in  the  region  of  the 
first  branch  of  the  tri-facial)  (Wallenberg,  Oppenheim,  Breuer-M arbur g, 
L.  R.  Miiller,  E.  Mutter). 

These  topographical  relations  explain  the  fact,  that  in  acute  bulbar 
paralysis,  as  a  result  of  focal  disease  in  the  medulla  oblongata,  the  hemi- 
anaesthesia  alternans  or  cruciata  frequently  appears  in  the  form  of  a  disso- 
ciated paralysis  of  sensation.  The  sensation  of  touch  alone,  which  can  prob- 
ably be  conducted  by  various  tracts,  is  rarely  affected.  Frequently  there  is  a 
loss  of  the  sensations  of  pain  and  temperature  in  the  trunk  and  the  extrem- 
ities on  the  side  opposite  the  lesion,  which  may,  however,  as  we  said  above, 
extend  also  to  the  upper  half  of  the  face  thus  forming  a  total hemianalgesia,  and 
which,  as  a  result  of  the  lesion  of  the  long  trigeminus  root,  is  connected  with  a 
disturbance  in  sensation  of  the  face  on  the  side  of  the  lesion.  Since  a  hypass- 
thesia  of  the  sensation  of  touch,  but  more  frequently,  a  loss  of  deep  sensibility 
on  the  side  of  the  lesion,  maybe  connected  with  it,  there  result  disease  pictures, 
which  have  a  certain  similarity  to  the  Brown-Sequard  type  of  unilateral  spinal 
lesion,  so  that,  in  connection  with  the  motor  disturbance  of  the  cerebral 
nerves,  which  appears  on  the  side  of  the  lesion,  especially  in  the  facialis,  one 
may  speak  in  a  similar  sense,  of  a  unilateral  bulbar  lesion. 

A  peculiar  type  in  this  respect  may  be  recognized  in  acute  bulbar  pa- 
ralysis, which  has  its  origin  in  thrombotic  occlusion  of  the  arteria  cerebelli 
inferior,  a  branch  of  the  vertebral.  Here  there  is  a  circumscribed  sharply 
denned  softening  in  the  lateral  section  of  the  medulla  oblongata  between  the 
olive,  the  fibrae  arcuatae  internae  and  the  restiform  bodies,  while  the  pyramids, 
the  lemniscus  and  the  floor  of  the  fourth  ventricle  are  often  spared;  the  tracts 
already  crossed  here,  for  the  conduction  of  the  pain  and  temperature  sensa- 
tions, the  (motor)  nucleus  ambiguus  vagi,  the  root  of  the  hypoglossus,  the 
spinal  trigeminus  root  and  the  restiform  bodies  are  affected.     The  symptoms 


ACUTE  BULBAR  PARALYSIS  331 

noted  correspond  to  such  a  lesion:  contralateral  hemianalgesia,  unilateral 
paralysis  of  the  musculature  concerned  in  swallowing  and  of  that  of  the 
palate,  larynx  and  tongue,  trigeminus  anaesthesia  on  the  side  of  the  lesion 
and  ataxia  of  the  extremities  on  the  same  side. 

Sometimes,  subjective  sensory  disturbances,  such  as  paresthesias  and 
perverse  temperature  sensations  (cold  is  felt  as  heat,  heat  as  cold)  make 
themselves  felt  in  the  anaesthetic  or  hypalgetic  extremities  in  acute  bulbar 
paralysis.  Homolateral  hyperaesthesia  has  been  also  observed  with  the  con- 
tralateral analgesia,  just  as  in  the  unilateral  spinal  lesion. 

Ataxic  disturbances  in  the  extremities  may  be  founded  upon  a  lesion  of  the 
kinaesthetic  tracts,  with  corresponding  loss  of  deep  sensibility.  They  appear 
more  frequently  in  the  form  of  cerebellar  ataxia,  associated  with  phenomena 
of  vertigo  and  falling  to  one  side,  in  lesions  of  the  restiform  bodies,  or  the 
tractus  spino-cerebellaris  lateralis  on  the  side  of  the  focal  injury,  and  then 
they  usually  remain  confined  to  a  disturbance  in  co-ordination  in  the  large 
joints,  or,  say,  the  musculature  of  the  trunk.  But  the  ataxia,  combined  with 
hypotonia,  may  be  so  severe  as  to  simulate,  at  first,  a  hemiplegic  paralysis. 
Nystagmus  (rotatorius) ,  too,  is  frequently  observed  as  sign  of  a  disturbance 
in  the  co-ordination  of  the  muscles  of  the  eye.  Disturbances  in  the  special 
sensory  organs  rarely  occur.  In  focal  injuries  that  are  situated  very  high, 
there  sometimes  appear  also  disturbances  in  sight  and  pupillary  phenom- 
ena, occasionally,  likewise,  disturbances  in  hearing,  when  the  nuclei  of  the 
auditory  nerve  are  affected.  Participation  of  the  vestibular  nuclei,  or  of 
the  appertaining  root  fibres,  then  also  of  the  cerebellum  and  its  connection 
with  the  pons,  bring  about  further  disturbances  in  the  harmonic  co-opera- 
tion of  the  muscles,  conditions  of  vertigo,  disturbances  in  equilibrium.  The 
disturbance  is  always  more  marked  on  the  side  of  the  lesion. 

Important  are  the  changes  which  point  to  a  participation  of  the  nervous 
centers,  which,  located  in  the  medulla  oblongata,  control  the  vegetative 
functions,  and  which  often  quickly  cause  an  unfavorable  turn  in  the  course 
of  the  disease.  As  such,  we  frequently  find  disturbances  in  respiration, 
dyspnoea  and  especially  periodic  breathing,  which  frequently  continues  for  a 
long  time  (Cheyne-Stokes)  with  occasional  alarming  pauses  in  respiration. 
Furthermore,  acceleration  or  irregularity  of  the  pulse  and  an  increase  in  the 
temperature  of  the  body,  which,  especially  when  the  outcome  is  fatal,  rises 
considerably  (even  as  high  as  1060  F.).  Albuminuria  and  glycosuria  occa- 
sionally accompany  the  disease  picture.  More  frequently  one  observes  a, 
flow  of  saliva.  Vaso-motor  disturbances  and  disturbances  in  the  secretion  of 
perspiration  occur  also. 

That  acute  bulbar  disease  is  capable  of  destroying  also  the  central 
connections  with  the  sympathetic,  is  shown  by  observation  of  the  occurrence 
of  Horner's  oculo-pupillary  symptom  complex  (ptosis,  enophthalmos  and 
miosis,  as  a  consequence  of  paresis  of  the  sympathetic)  on  the  side  of  the 


332  DISEASES  OF  THE  SPINAL  CORD 

central  focus  (E.  Midler).  Bladder  and  rectal  disturbances  (retention  of 
urine  and  feces)  are,  as  a  rule,  present  at  the  beginning  of  the  disease. 
Priapism,  likewise,  has  been  observed  in  connection  with  the  first  bulbar 
attack. 

Course  of  the  Disease  and  Prognosis. — In  many  cases  the  patients  die 
soon  after  the  first  attack;  large  hemorrhages,  many  small  multiple  hemor- 
rhages and  occlusion  of  the  basilar  artery  lead,  by  injury  of  the  important 
vital  bulbar  centers,  inevitably  to  death.  If  the  immediate  consequences  of 
bulbar  apoplexy  are  overcome,  life  is  still  threatened  by  the  results  of  the 
paralysis  of  deglutition,  which  appear  as  aspiration  pneumonia  and  bron- 
chitis and  cannot  bs  overcome,  because  of  the  insufficient  nutrition  and  the 
lowered  respiratory  function.  But  in  some  cases,  after  some  time,  through 
absorption  of  the  hemorrhages  and  restitution  of  the  circulation  in  the  injured 
parts,  the  phenomena  disappear  to  a  greater  or  less  extent,  and  in  time  there 
may  be  sufficient  restriction  of  the  anatomic  and  functional  defect,  so  that 
only  slight  motor  disturbance  remains.  In  slight  inflammatory  affections  of 
the  bulb,  even  complete  recovery  may  occur  in  young  patients,  in  spite  of  the 
phenomena  of  extensive  functional  loss  in  the  cerebral  nerves  and  the  ex- 
tremities at  the  beginning.  If  the  paralyses  persist,  the  phenomena  of  degen- 
erative atrophy  develop  more  and  more  distinctly  in  the  regions  of  the  nuclei 
that  have  been  attacked  (e.g.,  unilateral  wasting  of  the  tongue)  and  also  the 
spastic  phenomena  in  the  hemiparetic  extremities.  Nevertheless,  at  the 
beginning  of  the  disease  the  prognosis  is  dependent  on  the  extent  of  the  focal 
phenomena  and  on  the  severity  of  the  general  symptoms,  especially  the  impair- 
ment of  the  vegetative  functions. 

The  diagnosis  is  based  on  the  sudden  beginning,  or  at  least  on  the  unex- 
pected onset  associated  with  a  rapid  increase  of  the  symptoms  and  on  the 
arrangement  of  these  symptoms,  especially  the  combination  of  glosso- 
labio -pharyngeal  paralysis  with  alternating  hemiplegia  and  alternating  hemian- 
algesia.  From  pseudo-bulbar  paralysis,  acute  bulbar  paralysis  is  distinguished 
by  the  course  and  the  behavior  of  the  reflex  excitability  in  the  cerebral  nerves. 
Transitional  forms,  however,  occur. 

New  growths  in  the  medulla  oblongata  or  in  its  vicinity  often  induce, 
long  before  the  focal  symptoms  can  be  recognized,  symptoms  of  general 
brain  pressure;  the  same  is  true  of  cysticerci  and  chronic  meningitic  proc- 
esses; thereby  they  show  a  quite  different  course,  than  acute  bulbar  pa- 
ralysis, in  which  the  distal  effects  are  far  less  apparent  than  the  focal  phenom- 
ena, which  can  be  determined  with  great  exactness.  Bulbar  gliosis  also  can 
usually  be  excluded  without  any  difficulty,  by  considering  the  development 
of  the  symptoms;  it  must  be  remembered,  however,  that  occasionally 
through  intercurrent  hemorrhages  in  the  medulla  oblongata,  an  acute 
bulbar  paralysis  may  be  directly  induced  in  the  course  of  it.  In  multiple 
sclerosis,  the  pons  and  medulla  oblongata  seldom  remain  intact.     In  this 


PSEU DO-BULB AR  PARALYSIS  333 

disease,  the  total  disjunction  of  the  bulbar  centers  with  the  corresponding 
striking  and  well-defined  paralyses  and  disturbances  in  sensation,  etc., 
never  develops  as  quickly  as  in  bulbar  paralysis;  nay  more,  for  a  long  time, 
only  the  lighter  bulbar  functional  disturbances  appear,  together  with  a 
number  of  characteristic  spinal  and  cerebral  disturbances,  so  that  practically 
there  can  scarcely  ever  be  a  question  of  confusion  of  the  two,  if  a  thorough 
examination  is  made. 

Therapy. — Treatment  of  a  bulbar  apoplectic  attack  is  to  be  directed 
along  similar  fundamental  principles,  as  that  of  a  cerebral  apoplexy.  Abso- 
lute physical  and  psychical  rest  is,  of  course,  essential;  in  convulsive  attacks, 
it  may  be  attained  by  narcotics.  Local  or  general  blood-letting  is  to  be 
considered  only  when  hemorrhages  or  local  inflammation  are  suspected. 
The  possibility  of  a  syphilitic  aetiology  must  be  taken  into  consideration 
early  and  treatment  by  iodide  of  potassium  instituted;  in  case  of  disturbances 
in  swallowing,  injections  of  iodipin  are  always  advisable.  Nutrition  must 
be  carefully  watched  over  and  when  the  paralysis  of  swallowing  is  severe, 
is  most  satisfactorily  attained  from  the  beginning  by  means  of  a  stomach 
tube,  since  in  this  way,  too,  the  frequent  faulty  swallowing  and  therewith 
the  fear  of  aspiration  pneumonia  may  be  avoided.  In  unilateral  vagus 
paralysis  the  patients  are  able,  after  some  time,  to  swallow  more  easily 
again,  if  they  incline  their  head  towards  the  unparalyzed  side.  Cold  food 
is  more  easily  swallowed  than  warm  (crossed  hemithermanaesthesia) .  The 
analgesic  disturbances  demand  at  an  early  date  careful  protection  of  the 
patient  in  bed  (water  cushion)  and  marked  care  of  the  skin,  in  order  to 
avoid  bed-sores.  Protection  of  the  conjunctival  sclerae  and  the  cornea  in 
total  facial  paralysis  and  trifacial  anaesthesia  must  be  especially  considered. 
The  use  of  the  constant  current  may  assist  in  reviving  the  weakened  area 
of  the  motor  cerebral  nerves;  galvanization  at  the  nape  of  the  neck  is  espe- 
cially to  be  recommended  with  the  anode  and  excitation  of  the  movements 
of  deglutition.  If  the  paralyses  prove  after  longer  observation  to  be  constant 
and  irreparable,  with  unclouded  consciousness,  there  is  no  sense  in  with- 
holding from  the  severely  suffering  patient,  the  beneficent  action  of  effective 
narcotics. 

3.  Pseudo -bulbar  Paralysis 

The  bulbar-peripheral  centers  of  the  motor  cerebral  nerves  subserve 
as  do  the  spinal  motor  centers  of  the  gray  anterior  columns,  the  conduction 
of  cortico-fugal  motor  fibre  systems  coming  from  the  corresponding  centers 
of  the  psychomotor  region  of  the  central  convolutions  and  in  the  greater  part 
conducted  through  the  pyramidal  tracts.  They  transmit  the  motor  impulses 
from  the  cerebral  cortex  to  the  bulbar  nuclei  and  thereby,  for  the  most  part, 
bring  about  the  production  of  voluntary  movements  in  the  appertaining 


334  DISEASES  OF  THE  SPINAL  CORD 

muscular  regions  and  especially  in  the  extraordinarily  fine  shadings  in  the 
movements  of  the  lips,  pharynx  and  larynx  that  are  needed,  for  instance, 
in  speech.  Purely  reflex  movements,  partly  in  rather  complicated  movement 
complexes  (sucking  reflexes),  can  also  be  produced  in  the  bulbar  centers 
alone,  as  the  experience  in  the  malformations  caused  by  arrested  develop- 
ment in  the  cerebrum  teaches  us.  Then,  too,  the  involuntary  movements 
of  expression  and  emotion,  as  in  laughing  and  crying  can  be  evoked,  inde- 
pendent of  the  influence  of  the  cerebral  cortex,  by  the  aid  of  the  subcortical 
centers  in  the  lenticular  nucleus  and  the  optic  thalamus.  All  these  reflex 
movements,  which  in  early  childhood  are,  in  part  at  least,  of  great  physio- 
logical importance,  are  in  the  older  child  and  in  the  adult  subordinated  to 
the  restraining,  or  stimulating  control  and  the  inhibitory  influence  of  the 
cortex  of  the  brain;  in  part,  they  are  completely  crowded  out  by  the  pre- 
ponderance of  cortical  influence  under  normal  conditions.  A  destruction 
or  far-reaching  restriction  of  this  cortico-bulbar  influence  by  interruption 
of  the  supranuclear,  cortico-bulbar  tracts,  or  injury  of  the  corresponding 
centers  of  the  cortex,  will  therefore  be  followed  in  the  region  of  the  motor 
cerebral  nerves,  especially  in  the  musculature  of  swallowing,  of  the  lips, 
tongue  and  larynx,  by  a  paralysis  or  paresis  in  the  sense  of  the  loss  of  the 
ability  for  voluntary  movement  without  marked  atrophy,  while  reflex  excita- 
bility is  retained  and  under  certain  conditions  also  involuntary  emotional  and 
expressional  movements.  This  is  the  type  of  pseudo-bulbar  paralysis  in 
contra-distinction  to  acute  bulbar  paralysis  and  to  progressive  bulbar 
paralysis,  both  of  which,  though  differing  in  course,  location  and  extent  of 
lesion,  are  characterized  by  the  fact,  that  the  motor  nuclei  in  the  bulb  are 
themselves  injured  and  at  the  same  time  all  the  motor  bulbar  functions, 
even  the  reflex  and  automatic  ones,  corresponding  to  the  localization  of  the 
disease,  are  destroyed,  with  the  natural  consequence  of  a  degenerative 
atrophy,  if  the  duration  of  the  disease  is  sufficiently  long. 

It  is  significant,  for  the  pathological  physiology  of  pseudo-bulbar  paral- 
ysis, that  in  the  most  common  form  of  supranuclear  motor  disturbance,  cere- 
bral hemiplegia,  there  are,  as  a  rule,  no  symptoms  of  bulbar  paralysis;  it  is 
true  there  frequently  appears  a  contra-lateral  one-sided  paresis  of  the  lower 
(oral)  part  of  the  facial  nerve  and  of  the  tongue,  associated  with  the  paralysis 
of  the  extremities;  but  the  musculature  of  the  pharynx  and  larynx  is  spared. 
The  reason  for  this  is  that  these  muscles  receive  bilateral  cortical  innerva- 
tion, and  that  the  voluntary  movements  of  these  regions  on  both  sides  can  be 
sufficiently  supplied  from  one  hemisphere  alone.  The  cortico-bulbar  tracts 
must  therefore  be  injured  or  interrupted  in  both  hemispheres,  if  there  is  to 
be  a  clinically  recognizable  supranuclear  paralysis  of  the  pharyngeal  and 
laryngeal  muscles.  The  conditions  are  similar  as  regards  the  masticatory 
muscles,  those  of  the  forehead,  and  those  that  close  the  eyelids. 

^Etiology  and  Pathological  Anatomy. — Pseudo-bulbar  paralysis  occurs 


PSEUDO-BULBAR  PARALYSIS  335 

with  relative  frequency  in  childhood,  associated  with  more  or  less  promi- 
nent spastic-diplegic  phenomena,  in  those  cases  in  which  an  intrauterine  or  a 
later  acquired  inflammatory  or  traumatic  injury  caused  a  bilateral  extensive 
lesion  of  the  cortex  of  the  brain,  and  thereby  led  to  a  functional  disturbance 
of  the  psycho-motor  cortical  fields  of  the  bulbar  nerves  as  they  are  situated 
within  a  narrow  radius. 

In  adult  life,  it  is  principally  arteriosclerotic  changes  of  the  blood-ves- 
sels of  the  brain,  that  lead  to  multiple  hemorrhages  from  miliary  aneurisms, 
to  numerous  thrombotic  softenings,  and  to  extensive  degenerative  processes 
in  the  hemispheres,  and  thereby  to  bilateral  lesions  of  the  cortico-bulbar 
motor  systems  as  well  as  to  the  other  disturbances.  More  extensive 
apoplexies  appearing  consecutively,  in  both  halves  of  the  brain,  may 
have  the  same  effect.  Multiple  inflammatory  foci,  scleroses  and  embolic 
softenings,  occur  less  frequently  in  connection  with  this  condition.  Syphilis 
with  its  changes  in  the  blood-vessels  and  gummatous  focal  disturbances 
is  quite  frequently  a  cause  of  extensive  cerebral  disease. 

The  location  of  the  injury  may  vary.  The  focal  disease  is  rarely  restricted 
to  those  cortical  fields,  which  on  both  sides  direct  the  psycho-motor  stimu- 
lation of  the  bulbar  nerves  in  the  operculum.  More  frequently,  soften- 
ing foci,  cysts,  etc.,  may  be  demonstrated  in  the  medullary  layer  of  the  hemi- 
spheres, especially  in  the  posterior  parts  of  the  frontal  brain  or  even  lower 
down  in  the  radiation  of  medullary  fibres,  the  inner  capsule — in  the  region 
of  the  great  basal  ganglia.  The  cortical  lesion  of  one  side  may  combine 
with  a  focus  in  the  subcortical  medullary  layer  of  the  other  side  and  it  is 
characteristic  of  the  disease  that  is  based  mainly  upon  disturbances  in 
circulation,  that  the  arrangement  of  the  changes  in  the  brain  may  vary 
greatly. 

In  some  cases,  symptoms  of  pseudo-bulbar  paralysis  have  been  observed 
also  in  merely  one-sided  disease  of  the  brain.  It  is  a  matter,  then,  usually 
of  very  severe  lesions,  extensive  hemorrhages,  etc.,  in  which  a  functional 
injury  of  the  other  hemisphere  also  seems  understandable,  considering 
the  disturbances  in  pressure  and  circulation. 

If  the  atheromatosis  of  the  cerebral  arteries  has  progressed  so  far  that 
it  leads  to  multiple  or  extensive  focal  changes  in  both  hemispheres  of  the 
cerebrum,  it  does  not  seem  strange  that  the  more  delicate  branches  of  the 
vessels  in  the  brain  stem  and  in  the  medulla  oblongata  also  fail  to  remain 
intact  for  any  length  of  time  and  themselves  cause  changes  and  functional 
disturbances  in  the  nuclei  of  the  cerebral  nerves,  beside  the  cerebral  lesion 
of  the  supranuclear  system.  We  have,  then,  a  combination  of  pseudo- 
bulbar paralysis  with  phenomena  of  acute  genuine  bulbar  paralysis,  a 
disease  picture,  that  may  have  some  traits  of  both  forms,  cerebro-bulbar 
glosso-pharyngo-labial  paralysis  (Oppenheim) . 

Symptomatology. — The  cerebral  glosso-labio-pharyngeal  paralysis  is 


336  DISEASES  OF  THE  SPINAL  CORD 

but  rarely  ushered  in  by  a  severe  stroke.  More  frequently  there  is  observed 
a  subacute  progressive  development  of  the  disease  in  starts  or  jerks,  or  the 
symptom  complex  is  completely  developed  only  after  several  successive  apo- 
plectic attacks.  Thus  it  may  happen  that  after  a  first  attack,  a  hemiplegia 
appears  with  slight  disturbances  in  speech  and  swallowing,  the  latter 
two  disappearing,  while  the  hemiplegia  persists  and  that  only  after  a  second 
stroke  which  now  paralyzes  the  other  half  of  the  body  which  thus  far  had 
remained  intact,  associated  with  the  now  bilateral  injury  to  the  brain,  cere- 
bral-bulbar  paralysis  becomes  distinct.  In  other  cases  again,  the  disease 
may  develop  gradually  through  the  slow  accumulation  of  small,  multiple 
foci  of  cerebral  softening,  possibly  hastened  occasionally  by  slight  apoplecti- 
form attacks,  or  even  without  noticeable  phenomena. 

It  is  characteristic  of  the  anatomic  processes,  which,  in  connection  with 
diseases  of  the  blood-vessels,  underlie  the  functional  disturbances,  that  the 
phenomena  of  functional  loss  in  pseudo-bulbar  paralysis  are  not  strictly 
confined  to  a  definite  topographical  arrangement;  therefore,  they  do  not 
usually  appear  in  strict  symmetry  and  may  be  complicated  by  manifold 
focal  or  diffuse  cerebral  disturbances. 

Especially  characteristic  are  the  disturbances  in  the  facial  muscles  and 
in  those  concerned  with  chewing  and  swallowing,  likewise  in  the  musculature 
of  the  tongue,  palate,  and  larynx  with  the  prominent  phenomena  of 
dysarthria  and  dysphagia,  which,  as  a  rule,  are  combined  with  general  motor 
disturbances. 

The  exterior  appearance  of  the  patient  is  striking :  the  face  is  rigid,  immo- 
bile, the  mouth  half  open,  the  expression  lifeless  and  stupid.  The  continu- 
ous flow  of  saliva  has  a  still  more  disfiguring  effect.  The  posture  of  the 
body  is  stiff  and  awkward,  the  head  bowed  forward,  the  movements 
slow,  clumsy  and  spastic,  the  gait  a  laborious  drag  with  small  tripping 
steps. 

The  voluntary  movements  of  the  facial  muscles,  of  the  muscles  of  the  eye 
and  tongue  and  of  the  chewing  muscles,  are  very  markedly  limited,  or  entirely 
lost.  Speech  becomes  difficult  to  understand,  nasal,  or  thick,  due  to  the  dis- 
turbance of  articulation  and  of  the  formation  of  labial  sounds  and  to  the 
paralysis  of  the  soft  palate.  It  is  affected  likewise  in  intonation,  by  paresis 
of  the  laryngeal  muscles  and  disturbances  in  the  mechanism  of  breathing. 
The  difficulties  in  deglutition  but  seldom  attain  threatening  proportions,  but 
there  is  frequently  a  regurgitation  of  liquid  food  through  the  nose  and  solid 
food  frequently  gets  into  the  windpipe. 

The  muscles,  that  have  thus  been  withdrawn  from  the  dominion  of  the 
will  do  not  suffer  serious  atrophy,  as  a  rule,  even  after  prolonged  continuation 
of  the  disease,  the  lips  and  tongue  do  not  grow  thin,  show  no  fibrillary 
twitchings  and  neither  upon  indirect  or  direct  stimulation  do  they  give 
electric  reactions  of  degeneration. 


SYMPTOMATOLOGY  OF  PSEUDO-BULBAR  PALSY  337 

The  muscular  areas  that  voluntarily  can  not  be  set  in  motion  at  all,  or, 
only  to  a  very  slight  degree,  are  set  into  very  lively,  morbidly  increased 
activity  under  the  influence  of  emotional  disturbances,  or  rejiexly  by  cuta- 
neous and  sensory  stimulations. 

Thus  the  eyeballs,  remaining  ordinarily  in  rigid  immobility,  turn  involun- 
tarily in  the  direction  of  an  acustic  or  strong  optic  stimulation,  and  the 
eyeballs,  which  can  scarcely  be  moved  at  all  by  the  patient,  are  able  to  fol- 
low a  moving  object  by  reflex  adjustment  (Wernicke,  Oppenheim,  Strum- 
pell).  Very  slight  causes  evoke  spasms  of  crying  and  laughing,  in  swift, 
often  alternating  succession  (Oppenheim  and  Siemerling) .  At  the  same  time 
the  muscles,  which  are  almost  entirely  paralyzed  for  voluntary  movements, 
contract  strongly;  the  face,  formerly  immobile,  is  now  spasmodically  dis- 
torted; spasmodic  movements  of  expiration  and  coughing,  occasionally  a 
temporary  asphyctic  stoppage  of  breath  with  pronounced  cyanosis  of  the 
face,  accompany  these  peculiar  emotional  explosions,  in  which  the  more 
intelligent  patients  are  quite  conscious  of  the  insufficient  psychological 
motivation  of  these  more  reflex  emotional  and  expressional  disturbances, 
which  really  are  a  result  of  insufficient  cerebral  inhibition. 

A  permanent  increase  of  tension  in  the  paretic  muscles  of  the  face,  the 
tongue,  and  the  jaws  (trismus),  is  occasionally  present,  and  likewise  irrita- 
tion phenomena,  like  spasms  in  the  muscles  of  the  jaws,  gnashing  of  the 
teeth,  a  spasmodic  clearing  of  the  throat  and  choking  may  be  observed. 
The  insufficient  cerebral  restraint  and  inhibition  is  further  made  manifest 
in  an  increase  in  the  reflex  excitability  of  the  bulbar  nerves  and  in  the  appear- 
ance of  combined  reflex  movements,  which  under  normal  conditions,  early 
in  extrauterine  life  give  way  to  the  voluntary  use  of  the  labio-glosso-pharyn- 
geal  musculature.  The  masseter  reflexes  are  frequently  exaggerated,  and 
upon  tapping  the  lower  half  of  the  face,  reflex  contractions  appear  in  the 
orbicularis  oris,  recalling  sucking  movements  (Toulouse  and  Vurpas).  The 
palatal  and  pharyngeal  reflexes  are  retained.  Irritation  of  the  hard  palate 
causes  muscular  contractions  of  the  face  in  the  cheeks  or  upper  lip  (Henne- 
berg's  hard  palate  reflex).  Irritation  of  the  lips  or  of  the  oral  cavity  evokes 
rhythmic  movements  of  the  lips,  tongue,  lower  jaw,  and  in  the  throat  (eat- 
ing reflex  of  Oppenhein)  in  peculiar,  seemingly  almost  co-ordinated  purpose- 
ful combination.  Movements  of  swallowing,  even  when  there  is  disturbance 
in  consciousness,  may,  as  a  rule,  be  evoked  by  the  introduction  of  liquid  food. 
These  reflexes  appear  most  actively  in  infantile  pseudo-bulbar  paralysis, 
but  they  are  frequently  found  in  adults  also.  One  must  notice,  here,  that 
just  as  in  cerebral  hemiplegia,  the  reflex  increase  develops  to  its  greatest 
height  only  when  the  lesion  has  persisted  for  some  time,  and  the  subcortical 
or  bulbar  centers,  after  the  interruption  of  the  cerebral  influence  have,  to 
some  extent,  recovered,  or  again  become  accustomed  to  independent 
activity. 


338  DISEASES  OF  THE  SPINAL  CORD 

That  the  control  of  respiratory  innervation  may  suffer  also  is  proven, 
not  only  by  the  cases  of  the  disturbances  in  breathing  in  obsessional  laughing 
and  crying,  but  by  the  frequently  occurring  conditions  of  dyspnoea  and 
periodic  breathing  (Cheyne-Stokes)  and  the  difficulty  of  regulating  breathing 
during  speaking  and  swallowing  (Hartmann).  Acceleration  and  irregu- 
larities of  the  heart's  action  also  occur.  In  greater  impairment  of  the  auto- 
matic functions  of  the  bulbar  centers  (increase  in  temperature,  glycosuria, 
etc.),  however,  one  must  consider  that  the  arterio-sclerotic  disease  may 
likewise  occasion  foci  in  the  pons  and  the  medulla  oblongata,  in  addition 
to  the  cerebral  injuries,  whereby  a  disease  picture  develops,  which  no  longer 
exactly  fits  the  frame  of  supranuclear  bulbar  paresis. 

As  a  rule,  in  pseudo-bulbar  paralysis,  there  are  also  spastic  motor  dis- . 
turbances  in  both  arms  and  legs,  corresponding  to  the  bilateral  cerebral 
injury,  or,  according  to  the  localization  of  the  cerebral  foci,  only  in  the 
form  of  a  cerebral  hemiplegia.  In  extensive  arterio-sclerosis  of  the  nervous 
system,  the  spastic  character  of  the  paresis  of  the  extremities  may  be  more  or 
less  obliterated  or  concealed  by  injury  of  the  compensation  tracts  and  of 
the  long  and  short  reflex  arcs.  Occasionally,  too,  in  a  restriction  of  the 
central  disease  to  the  centers  and  supranuclear  tracts  of  the  motor  cere- 
bral nerves,  there  appears  a  pseudo-bulbar  paralysis  with  but  very  slight 
movement  disturbances  in  the  limbs  (senile  abasia)  (Naunyn)  or  without 
any  at  all. 

Of  other  complications  of  cerebral  labio-glosso-pharyngeal  paralysis, 
we  must  lay  stress  upon  the  very  frequent  psychic  disturbances,  which  make 
themselves  manifest  especially  as  intellectual  weakness,  deterioration  in  the 
ability  to  receive  and  retain  new  impressions,  and  in  memory,  and  may  be 
increased  to  disturbances  in  orientation,  clouded  intellect,  conditions  of 
delirious  excitement.  Of  course  one  must  beware  of  deciding  on  the  pres- 
ence of  a  psychic  defect,  from  the  external  aspect  alone — from  the  com- 
bination of  a  staring  expression  in  the  face  with  an  open  mouth,  dysarthric 
speech  disturbances  and  general  difficulty  in  movement.  Furthermore, 
the  disease  picture  may  be  variously  enlarged  by  a  series  of  cerebral  dis- 
turbances corresponding  to  the  extension  of  the  injury  in  the  brain:  motor 
and  sensory  aphasia,  hemianopsia  and  other  central  visual  and  auditory 
disturbances,  disturbances  in  equilibrium,  as  well  as  more  or  less  extensive 
disturbances  in  sensation,  especially  of  the  sense  of  touch  and  localization, 
but  also  hemianaesthesia  and  disturbances  in  co-ordination.  Among  motor 
symptoms,  athetosis  and  chorea  from  central  irritation  either  on  one  or 
both  sides  are  sometimes  seen.  Pupillary  disturbances  and  optic  atrophy 
have  been  occasionally  observed  along  with  the  bulbar  symptoms  of  func- 
tional loss. 

More  frequently  and  especially  in  the  beginning  of  the  disease,  but 
also  as  a  permanent   concomitant  phenomenon,  pseudo-bulbar  paralysis 


PSEU DO-BULB AR  PARALYSIS  339 

causes  a  loss  of  the  voluntary  control  over  the  functions  of  the  bladder  and 
rectum,  which  is  present  chiefly  in  the  form  of  retention  of  feces  and  urine, 
but  may  lead  also  to  enuresis  and  coprostasis. 

The  diagnosis  of  pseudo-bulbar  paralysis  will  offer  no  great  difficulties 
if  there  is  complete  development  of  the  supranuclear  bulbar  paralysis  with 
retained  muscular  nutrition  and  electrical  excitability,  with  normal,  even 
increased  reflex  and  emotional  motor  excitability  and  with  the  frequent 
complications  of  psychic  disturbances  and  cerebral  paralysis  of  the  extrem- 
ities. A  confusion  with  progressive  bulbar  paralysis  is  excluded  by  the 
fact  that  this  has  a  far  more  insidious  course,  and  is  distinguished  by  degen- 
erative atrophy,  fibrillar}'  tremors  and  loss  of  electrical  excitability,  as  well 
as  of  the  reflexes  of  the  motor  cerebral  nerves  and  by  the  strict  confinement 
to  these  areas  with  the  exception  of  the  nerves  of  the  eye  muscles.  Acute 
apoplectic  bulbar  paralysis  may  occasionally  be  mistaken  for  the  pseudo-bul- 
bar paralysis,  but  can  usually  be  distinguished  from  it  by  the  presence  of 
genuine  bulbar  focal  symptoms,  especially  if  alternating  hemiplegia  and 
alternating  hemianalgesia  exist.  Amyotrophic  lateral  sclerosis  with  bulbar 
localization  can  give  rise  to  disturbances  in  movement,  which  resemble 
closely  those  of  pseudo-bulbar  paralysis,  but  it  will  make  itself  known  by  the 
steadily  progressing  course  without  apoplectic  advances  by  fits  and  starts, 
by  the  occurrence  of  degenerative,  localized  muscular  atrophies  and  by 
sparing  of  the  psyche  and  all  functions  not  purely  motor.  In  myasthenic 
bulbar  paralysis,  the  phenomena  of  fatigue,  weakness  and  exhaustion, 
beside  the  electro-diagnostic  signs,  predominate  in  the  disease  picture,  and 
it  wholly  lacks  the  phenomena,  so  characteristic  of  pseudo-bulbar  paralysis, 
of  involuntary  motor  processes  excitable  by  reflexes,  frequently  increased 
to  veritable  paroxysms  in  the  paretic  bulbar  muscles. 

The  prognosis  of  pseudo-bulbar  paralysis  is  unfavorable,  even  when 
one  considers  only  the  excitant  factor,  the  atheromatosis  of  the  cerebral 
arteries.  The  recurrence  of  apoplectic  seizures  and  therewith,  a  dangerous 
turn  in  the  disease,  is  always  to  be  feared,  while  thanks  to  the  reflex  activity 
of  the  bulbar  centers,  there  is  usually  no  danger  to  life  directly  from  disturb- 
ance in  deglutition  and  aspiration  of  foreign  bodies.  With  careful  treatment, 
avoidance  of  all  influences,  that  might  suddenly  increase  the  pressure  of  the 
blood  (excitement,  alcohol,  caffein)  the  patients  may,  under  certain  con- 
ditions, be  kept  alive  for  a  comparatively  long  time;  with  careful  attention 
even  an  improvement  in  the  disturbances  is  possible.  Treatment  by  iodide 
of  potassium  is  always  to  be  recommended;  if  a  syphilitic  aetiology  is  suspected, 
or  proved  by  anamnestic  or  objective  signs  (scars,  Wasserman's  serum 
reaction,  etc.),  a  well-directed  combined  treatment  of  iodide  of  potassium 
and  mercury  may  cause  a  considerable  improvement  in  the  disturbance 
and  in  any  case  effectively  combat  the  further  spread  of  the  disease  in  the 
cerebrum  and  possibly  in  the  bulb  as  well. 


34o  DISEASES  OF  THE  SPINAL  CORD 

3.  MULTIPLE  INSULAR  SCLEROSIS.     (Disseminated  Sclerosis) 

BY 

HERMANN  SCHLESINGER  (Vienna) 
^Etiological  Factors 

The  disease  is  traced  back  by  a  number  of  authors  to  early  (embryolog- 
ical)  disturbances  and  a  disposition  upon  the  part  of  the  neurogliar  tissue 
to  proliferate  (Striimpell,  Mueller) .  I  am  inclined  to  agree  with  Oppenheim 
that  congenital  anomalies  or  such  developmental  disturbances  as  begin  very 
early  in  life  represent  only  a  factor  favoring  the  special  disposition  for  the 
disease. 

Marie  is  of  the  opinion  that  acute  infectious  diseases  represent  probably 
the  most  important  setiological  factor.  The  same  view  is  held  by  many  of  the 
later  writers,  as  Kahler  and  Pick  (even  before  Marie),  Oppenheim,  Maixner, 
Redlich,  Spiller  and  others.  From  my  own  experience  I  should  regard  this 
as  the  cardinal,  the  causal  factor. 

The  diseases  concerned  chiefly  are  typhoid,  pneumonia,  diphtheria, 
influenza,  scarlatina,  variola.  Malaria  also  has  been  observed  rather 
frequently  as  an  setiological  factor.  Intoxications  (mercury,  carbonic 
oxide,  tin,  possibly  manganese  also),  may  call  forth  the  disease. 

The  onset  of  the  disease  has  frequently  been  observed  after  severe 
psychic  disturbances  and  especially  after  colds ;  furthermore  also  after  severe 
traumatisms  (Schlagenhaufer,  Mendel,  Redlich).  In  a  great  many  cases  no 
setiological  factors  can  be  discovered.  The  period  of  adolescence  is  particu- 
larly predisposed  to  the  disease. 

Anatomic  Changes 

Disseminated  sclerosis  is  characterized  by  the  appearance  of  multiple 
lesions  in  the  brain  and  spinal  cord.  The  number  of  foci  may  range  from 
a  few  up  to  several  hundred.  Their  distribution  is  not  regular;  sometimes 
with  a  relatively  large  number  of  foci  in  the  spinal  cord,  there  are  only 
a  few  in  the  brain.  They  may  affect  every  section  of  the  central  nervous 
system,  are  occasionally  arranged  with  striking  symmetry  and  show  quite 
considerable  variations  in  size.  The  largest  foci  are  found  in  the  pons  and 
in  thejbrain.  The  optic  nerve  and  the  chiasma,  also,  often  have  one  or 
more'.foci. 

The  foci  themselves  are,  as  a  rule,  very  strictly  circumscribed  and  even 
macroscopically  are  clearly  distinguished  from  their  surroundings  by  their 
gray  color.  They  are  recognizable,  if  situated  near  the  surface,  even  before 
cutting  into  the  spinal  cord,  and  may  be  clearly  felt  by  the  palpating  finger 
as  harder  spots.  They  may  lie  partly  in  the  white  and  partly  in  the  gray 
matter. 


MULTIPLE  INSULAR  SCLEROSIS  341 

In  the  foci,  one  finds  upon  microscopic  examination  no,  or  only  a  few 
nerve  fibres  retaining  their  medullary  sheath.  But  many  well-preserved 
axis  cylinders  pass  through  the  foci.  The  neurogliar  tissue  is  extraordinarily 
increased,  and  forms  a  thick  felt-like  layer,  which  does  not  show  any  ten- 
dency to  softening  anywhere.  The  ganglion  cells,  even  in  the  center  of  the 
foci,  are  not  injured,  but  the  vessels  often  show  grave  changes;  their  walls 
are  thickened  and  the  lumen  narrowed,  or  even  entirely  obliterated.  Sec- 
ondary degenerations  are  absent,  even  after  long  continuation  of  the  disease, 
and  with  numerous  foci.  Upon  the  advent  of  diffuse  sclerosis,  changes  in 
the  form  of  the  sections  attacked,  especially  in  the  pons,  occasionally  occur. 

Besides  the  old  foci,  one  frequently  sees  diseased  spots  in  which  the  proc- 
ess is  of  more  recent  date;  small-celled  infiltration  of  the  vessels  (filling  of 
the  adventitial  lymphatic  spaces  with  fatty  granular  cells)  is  exhibited; 
processes  of  breaking  down  in  the  focal  regions  are  still  going  on.  Foci  of 
more  recent  date  are  found  especially  in  cases  with  an  acute  and  subacute 
course. 

The  pathogenesis  is  by  no  means  clear.  The  various  views  are,  in  part, 
in  direct  contrast  with  one  another.  Some  authors,  like  Schmaus  and  E. 
Miiller  speak  of  congenital  conditions  (hypoplastic  conditions  of  the  nervous 
system).  Other  investigators  consider  proliferation  of  the  neuroglia  the 
primary  change  (Rossolimo,  Strilmpell,  Probst) .  Still  others  believe  in  a  pri- 
mary lesion  of  the  parenchymatous  parts  of  the  nervous  system  (Redlich, 
Koppen).  The  disturbance  of  the  vascular  apparatus  is  considered  as  a 
causal  factor  especially  by  Taylor,  Rindjleisch,  Ribbert.  The  theory  of  an 
inflammatory  origin  finds  many  supporters  (Charcot,  Erb,  Leyden,  Oppen- 
heim).  It  is  very  probable  that  an  acute  disseminated  encephalomyelitis, 
and  in  particular  that  form  accompanied  by  cellular  infiltration  (Oppenheim), 
leaves  in  its  wake,  as  a  resulting  condition,  a  multiple  sclerosis. 

Symptoms  and  Course 

We  are  indebted  to  an  excellent  study  by  Charcot  for  our  knowledge 
of  several  cardinal  symptoms  of  multiple  sclerosis,  without  which  for  a  long 
time  one  could  scarcely  venture  to  make  a  strict  diagnosis  of  this  affection. 
Not  for  some  time  afterwards  were  quite  a  number  of  other  symptoms  dis- 
covered. In  the  early  stages  of  the  disease  cerebral  or  spinal  symptoms 
may  occupy  the  foreground.  Very  frequently,  the  disturbances  begin  with 
visual  disturbances  as  has  been  pointed  out  particularly  by  Oppenheim, 
Bruns,  Stoelting  and  Uhthof}.  Ophthalmoscopic  examination  then  discloses 
a  neuritis,  or  an  optic  atrophy. 

These  symptoms  may  be  the  first  ("like  vanguards")  (Oppenheim),  and 
are  therefore  very  often  misinterpreted.  Since  our  knowledge  of  multiple 
sclerosis  has  been  steadily  increasing,  it  happens  more  and  more  frequently, 
that  a  " retro-bulbar  neuritis"  afterwards  turns  out  to  have  been  a  precursor 


342  DISEASES  OF  THE  SPIXAL  CORD 

of  multiple  sclerosis.  I  have  seen  quite  a  few  patients,  in  which  this 
was  the  first  symptom  of  the  disease;  occasionally  it  precedes  the  other  phe- 
nomena by  years.  In  other  cases,  a  uni-  or  bilateral  optic  atrophy  develops 
very  slowly  or  very  quickly,  with  a  peculiar  gray  discoloration  of  the  disc: 
this  optic  atrophy  as  a  rule  is  partial  (Uhthoff),  more  frequently  temporal 
than  nasal,  rarely  ever  total,  and  was  found  in  almost  half  the  cases.  Since 
the  visual  disturbances  in  the  great  majority  of  cases  are  transient  and  are 
not  associated  with  pupillary  disturbances,  they  are  frequently  regarded  as 
hysterical  precisely  because  of  their  instability,  when  there  has  been  no  ex- 
amination of  the  fundus.  Bruns-Stoelting  places  the  frequency  of  cases 
beginning  with  optic  atrophy  at  30%.  Even  very  marked  visual  disturb- 
ances may  disappear.  One  of  my  patients,  who,  five  years  ago,  was  unable 
to  recognize  fingers,  now  reads  the  finest  print  without  difficulty. 

Paralyses  of  the  muscles  of  the  eye,  especially  of  those  supplied  by 
the  oculomotor  and  the  abducens,  are  frequent  in  the  early  stage.  Ophthal- 
moplegia is  very  rare — -as  a  rule,  only  a  few  of  the  muscles  are  disturbed 
and  the  internal  muscles  of  the  eye  remain  normal.  Often  the  paralysis 
affects  the  associated  movements.  Permanent  paralyses  are  rare.  After  a 
shorter  or  longer  time,  the  paralysis  of  the  muscles  of  the  eye  disappears. 
Difference  in  the  size  of  the  pupils  is  common,  reflex  rigidity  very  rare. 
Charcot' s  cardinal  symptom,  nystagmus,  is  observed  quite  frequently.  First 
visible  in  movements,  in  which  the  already  paralyzed  muscles  are  used 
("nystagmoid  twitchings")  it  becomes  more  distinct  later  on  and  is  notice- 
able even  when  the  eyes  are  at  rest.  Nystagmus  horizontalis,  as  well  as 
nystagmus  verticalis  and  rotatorius  may  be  observed;  the  nystagmus  is 
most  frequent  when  the  patient  looks  to  one  side.  Redlich  regards  it  as 
completely  analogous  to  intention  tremor. 

Paralytic  weakness  of  one  extremity,  especially  of  one  leg,  has  often 
been  observed  as  an  initial  symptom.  I  have  observed  at  the  same  time 
three  cases  with  initial  paresis  of  one  extremity  (twice  of  a  leg,  in  one  case 
of  an  arm).  In  addition  to  the  weakness,  rigidity  of  the  musculature  is 
usually  also  present. 

The  presence  of  a  spastic  paralysis  in  the  lower  extremities,  is  quite 
frequently  observed  in  multiple  sclerosis.  Charcot  already  pointed  to 
this  and  later  observers  have,  for  the  most  part,  confirmed  it.  Occasion- 
ally this  condition  is  only  suggested  and  recognized  with  difficulty — in  other 
cases,  it  is  well  developed. 

Increase  of  the  patellar  reflexes  and  ankle  clonus  belong  to  the  most  fre- 
quent and  important  early  symptoms  of  disseminated  sclerosis.  The 
increase  in  the  reflexes  may  be  demonstrated  on  both  sides.  In  the  early 
stages  also  tremor  in  one  extremity  is  not  rare. 

If  the  disease  is  fully  developed,  the  cardinal  symptoms,  postulated  by 
Charcot,  are  present — nystagmus,  intention  tremor,  scanning  speech,  to  which 


MULTIPLE  INSULAR  SCLEROSIS  343 

are  further  added  reflex  exaggeration  in  the  lower  extremities  with  loss  of 
the  abdominal  reflex. 

The  so-called  intention  tremor  is  a  coarse  trembling  of  the  extremities 
while  performing  voluntary  movements;  the  trembling  becomes  more  and 
more  pronounced,  the  nearer  the  extremity  approaches  the  goal  which  it  is 
endeavoring  to  reach.  At  the  same  time,  muscle  groups  lying  at  some  dis- 
tance are  contracted.  If  in  a  relatively  severe  case,  with  the  patient  in 
a  sitting  posture,  he  attempts  a  movement  with  one  extremity,  his  entire 
body  begins  to  tremble  (wave  to  and  fro).  If  the  patient  takes  hold  of  an 
object,  it  is  swung  to  and  fro  with  the  extremity,  but  usually  held  fast,  in 
as  much  as  intention  tremor  is  never  associated  with  any  considerable 
weakness  of  the  extremities.  The  number  of  the  contractions  is  not  very 
large,  much  smaller  than  in  Basedow's  disease. 

The  head  also  takes  part  in  the  oscillating  movements.  In  more  severe 
cases,  the  tremor  in  the  trunk  becomes  very  decided  and  during  intended 
walking  the  trembling  of  the  legs  is  also  very  distinct  in  most  of  the  cases. 
The  tremor  does  not  stop,  even  if  the  movements  are  frequently  performed 
in  succession,  but  the  intention  tremor  disappears  altogether,  when  the 
patient  is  in  repose. 

Scanning  speech  is  a  very  noticeable  disturbance.  The  speech  becomes 
monotonous  and  its  capacity  for  modulation  is  absent.  It  becomes  drawn 
out  as  each  syllable  is  clearly  separated  from  the  one  following.  Even 
though  this  speech  disturbance  sometimes  appears  early,  in  the  majority 
of  cases,  scanning  speech  is  a  symptom,  that  becomes  pronounced  only 
at  the  height  of  the  disease.  Brady phasia  is  occasionally  present  without 
synchronous  scanning  (Redlich).     Dysarthria  is  no  rare  symptom. 

The  exaggeration  of  the  tendon  reflexes  has  already  been  mentioned. 
An  important  symptom  is  the  diminution  of  the  cutaneous  reflexes,  especially 
of  the  abdominal  reflexes  (Mueller) ,  which  may  appear  early. 

Besides  the  increase  of  the  reflexes,  especially  in  the  lower  extremities, 
other  symptoms  as  well  point  to  lesions  of  the  pyramidal  tracts.  Thus  the 
Babinski  phenomenon  is  present,  and  dorsal  flexion  of  the  great  toe  appears 
not  only  when  the  test  is  made  in  the  typical  way  (stroking  the  inner  margin 
of  the  foot  or  the  instep  or  above  the  metatarsus  quintus),  but  sometimes 
even  in  walking,  if  the  patient  sets  his  foot  down  firmly.  OppenheinCs 
leg  phenomenon  is  clearly  developed  (dorsal  flexion  of  the  great  toe  upon 
vigorous  stroking  of  the  edge  of  the  tibia) .  The  testing  for  Mendel's 
reflex  upon  the  dorsum  of  the  foot  occasionally  elicits  plantar  flexion  of  the 
great  toe  by  tapping  the  dorsum  of  the  foot  at  the  base  of  the  third  and 
fourth  metatarsal  bones  (whereas  in  healthy  people,  a  dorsal  flexion  results). 

Disturbances  in  sensation  are  more  frequent  in  the  course  of  the  disease, 
than  was  formerly  thought.  Disturbances  in  perception,  however,  are  only 
temporary,  and  severe  only  in  exceptional  cases. 


344  DISEASES  OF  THE  SPINAL  CORD 

Phenomena  appear  due  to  sensory  irritation,  especially  intercostal  pains, 
pains  in  the  joints,  paresthesias,  and  neuralgic  troubles.  In  one  of  my  cases 
neuralgia  of  terrible  severity  persisted  for  weeks.  Oppenheim  reports  the 
case  of  a  patient  in  whom  "tic  douloureux"  was  an  initial  and  permanent 
symptom  of  the  multiple  sclerosis. 

Sensory  phenomena  of  functional  loss  usually  do  not  persist  for  any 
length  of  time.  They  are  not  very  serious  and  cause  only  a  deadening  of 
sensibility,  no  complete  loss  of  sensation.  The  sensory  disturbance  may  be 
uniform  and  affect  the  various  qualities  of  (superficial  as  well  as  deep)  sen- 
sation in  the  same  way,  or  there  may  develop  a  partial  disturbance  in  sensa- 
tion, which  affects  only  the  pain  or  temperature  sense  and  disappears  after 
a  shorter  or  longer  duration  (Freund).  They  may  then  return  anew  and 
disappear  again.    Permanent  disturbances  of  sensation  are  rare. 

Oppenheim  observed  the  Brown-Sequard  symptom  complex  as  a  transient 
phenomenon  and  also  hemianesthesia. 

Paresthesias,  like  formication,  feeling  of  numbness,  are  not  very  rare. 

Rectal  disturbances  are  observed  at  the  beginning  of  the  disease  only  in 
exceptional  cases.  In  the  late  stage  of  the  affection  incontinentia  alvi  is 
frequently  seen.  I  have  observed  it  in  quite  a  large  number  of  cases.  The 
patients,  as  a  rule,  perceive  the  passage  of  the  feces,  but  can  not  prevent  it. 
I  have  often  seen  this  as  a  transient  symptom. 

Bladder  disturbances  are  frequent,  v.  Frankl-Hochwart  and  Zuckerkandl 
found  them  in  four-fifths  of  their  cases,  especially  when  paraplegia  of  the 
lower  extremities  existed.  They  may  be  the  very  first  signs  of  the  disease, 
which  is  followed  by  others  only  after  months,  or  even  years.  The  form 
of  the  bladder  disturbances  is  not  always  the  same.  At  one  time  simple 
incontinence  with  dribbling  of  the  urine  appears;  at  another,  spasms  of  the 
sphincter  with  intact  sensibility  of  the  bladder,  which  demands  catheteriza- 
tion, or  there  develops  the  "irritable  bladder"  with  frequent  tenesmus  and 
incomplete  emptying  and  a  sudden  shifting  between  incontinence  and 
retention. 

The  bladder  disturbances  may  disappear  permanently  or  return  after 
a  shorter  or  longer  time.  Persistence  of  the  bladder  disturbances  is  noted, 
as  a  rule,  only  towards  the  end  of  the  disease. 

Bladder  disturbances  are  more  frequent  than  rectal  disturbances  accord- 
ing to  my  observations,  and  affect  many  patients  in  the  initial  stage  of  the 
disease. 

Disturbances  in  the  realm  of  the  sexual  sphere  are  much  less  frequent 
than  might  be  suspected,  considering  the  frequency  of  the  bladder  disturb- 
ances. Only  patients  with  far  advanced  disseminated  sclerosis  complain 
of  frequent  pollutions  or  unsuccessful  cohabitation.  One  of  my  patients 
married  some  years  after  the  beginning  of  his  disease,  and  begot  a  goodly 
number  of  children. 


SYMPTOMATOLOGY  OF  MULTIPLE  INSULAR  SCLEROSIS     345 

In  some  cases,  bulbar  disturbances  develop.  Sudden  paralyses  of  the 
soft  palate,  of  the  pharyngeal  musculature  of  transitory  character  are  thus 
explained.  Disturbances  in  deglutition  and  mastication,  regurgitation 
through  the  nose,  faulty  swallowing,  may  be  present  as  initial  symptoms, 
but  do  not  remain.  Disturbances  of  phonation  may  be  caused  by  paresis 
of  the  laryngeal  musculature,  and  it  is  especially  pareses  of  the  interni, 
rarely  paralyses  of  the  vocal  cords,  that  are  observed.  Trembling  of  the 
vocal  cords  in  phonation  has  likewise  been  described.  Oppenheim  describes 
tremolo  of  the  voice  in  uttering  a  (German  E). 

Like  other  motor  paralyses  in  disseminated  sclerosis,  the  bulbar  paralyses 
do  not  persist;- 1  myself  have  seen  the  most  severe  bulbar  processes  retro- 
gress. Towards  the  end  of  the  disease,  these  phenomena  may  become 
permanent  and  present  themselves  in  a  form  clinically  resembling  progress- 
ive bulbar  paralysis,  but  without  muscular  atrophy  in  the  region  of  distri- 
bution of  the  bulbar  nerves. 

Obsessional  laughing  and  crying  are  sometimes  observed  very  early.  If 
they  exist,  they  sometimes  persist  permanently.  One  of  my  patients  was 
first  attacked  by  obsessional  laughing  at  a  funeral,  as  he  stepped  up  to  the 
open  grave,  and  has  now  suffered  for  ten  years,  uninterruptedly,  from  this 
very  annoying  symptom.  The  transition  from  compulsory  laughing  to 
compulsory  crying  may  take  place  very  suddenly. 

Apoplectiform  attacks,  which  threaten  the  patient  in  every  stage  of  the 
disease,  but  luckily,  too,  spare  many  patients,  play  an  important  part.  The 
attacks  are  occasionally  accompanied  by  complete  loss  of  consciousness, 
and  are  then  accompanied  in  some  cases  by  epileptiform  seizures.  Or,  it 
may  be  that  only  severe  attacks  of  vertigo  occur,  which  may  be  compared 
to  apoplectiform  attacks,  strike  the  patient  suddenly,  and  if  he  is  standing 
or  walking  at  the  time  of  the  seizure  throw  him  to  the  ground.  In  direct 
connection  with  these  attacks,  one  sees  more  or  less  extended  unilateral  pa- 
ralyses of  the  cerebral  nerves.  Or  immediately  after  the  attacks,  combinations 
of  cranial-nerve  palsies  will  be  observed,  which  show  extraordinary  variations. 
All  the  cerebral  nerves,  even  the  auditory,  are  affected  occasionally.  Hemi- 
ataxia  after  an  attack  has  been  described  by  Oppenheim,  who  reports, 
too,  rarer,  suddenly  occurring  paralyses  (atrophic  paralysis  with  ataxia 
of  one  arm).  I  have  seen  the  clinical  picture  of  an  acute  extended 
bulbar  paralysis  with  paralysis  of  deglutition,  of  the  soft  palate  and  of  the 
larynx. 

Ataxia  is  occasionally  present.  In  such  instances  it  affects  either  only 
single  extremities,  or  one-half  of  the  body,  or  finally,  only  the  lower  extrem- 
ities. It  is  of  the  nature  of  cerebellar  ataxia  (Redlich),  and  manifests  itself 
by  a  tottering  gait.  According  to  Oppenheim,  ataxia  must  be  assumed  to 
be  present  if  the  disturbance  increases  when  the  eyes  are  closed.  Ataxia 
does  not  by  any  means  exclude  the  existence  of  spastic  conditions  in  the 


346  DISEASES  OF  THE  SPIXAL  CORD 

limbs.  Its  acute  development  in  the  upper  limbs  with  or  without  bulbar 
symptoms  has  been  observed  several  times. 

Disturbances  of  the  intellect,  especially  failing  memory,  are  common; 
delirium,  and  dementia  are  present  only  in  exceptional  cases;  if  either 
occurred,  they  were,  as  a  rule,  in  most  of  my  cases,  transitory  phenomena. 

Complaints  about  headaches  are  common  and  they  occasionally  resemble 
migraine. 

Respiratory  disturbances  are  unusual.  Towards  the  end  of  life  Cheyne- 
Stokes  respiration  is  observed.  Oppenheim  has  described  conditions  of 
asphyxia. 

The  pulse  rate  is  accelerated,  if  a  bulbar  lesion  is  present;  tachycardia 
is  often  continuous,  but  remains  within  moderate  bounds. 

Muscular  atrophies  are  very  rare.  Should  they  develop,  however,  the 
electrical  excitability  is  changed  only  in  exceptional  cases.  Atrophy  is  seen 
in  individual  groups  of  muscles,  or  possibly  in  the  musculature  of  an  entire 
limb. 

The  paresis  usually  affects  both  legs,  so  that  spastic  spinal  paralysis 
results.  As  a  matter  of  fact,  in  many  spastic  spinal  paralyses,  the  presence 
of  a  disseminated  sclerosis  is  only  established  postmortem  (as  long  as  these 
peculiarities  were  unknown).  Unilateral  forms  with  paralytic  phenomena 
and  tremor  in  one-half  of  the  body  were  observed  by  Bikeles,  Oppenheim, 
Edwards.  Extensive  atrophic  pareses,  concerning  which  Probst  reports,  are 
verv  rare.  In  his  case,  the  symptom  complex  of  amyotrophic  lateral 
sclerosis  was  present. 

Course. — With  respect  to  the  course,  three  forms  may  be  distinguished: 
i.  Forms  with  an  insidious  onset.  2.  Cases  with  acute  initial  symptoms,  but 
slow  course  with  remissions  and  exacerbations.  Both  forms  appear  pre- 
ponderantly in  young  persons.  3.  Acutely  beginning  and  quickly  ending 
cases  ("acute  multiple  sclerosis"). 

The  cases  with  an  insidious  onset  seem  to  be  the  more  frequent.  Since 
the  symptoms  develop  very  gradually  and  show  a  considerable  tendency 
to  remissions,  the  disease  in  most  cases  is  supposed  to  be  functional.  This 
error  is  supported  by  the  fact  that  the  disease  is  nearly  always  combined 
with  hysteria. 

In  the  discussion  of  the  individual  symptoms,  the  possibility  of  an  acute 
onset  was  mentioned  several  times.  With  this  onset  the  initial  picture 
may  be  very7  varied  and  paralyses  of  the  most  varied  kinds  may  develop 
(unilateral  paralysis  of  the  body,  ataxia  of  single  limbs,  bladder  and  rectal 
disturbances,  sensory  paralyses  and  those  of  the  muscles  of  the  eye,  bulbar 
paralyses,  etc.). 

Extraordinarily  important  for  the  recognition  of  the  nature  of  dissem- 
inated sclerosis  is  the  peculiarity  of  the  disease,  in  having  long  remissions 
and  in  advancing  intermittently. 


MULTIPLE  INSULAR  SCLEROSIS  347 

The  remissions  may  be  surprising  even  in  advanced  cases,  and  in  less 
progressive  ones  create  the  impression  of  a  cure. 

Exacerbations  follow  especially  under  the  influence  of  weakening  diseases, 
of  the  puerperium,  great  emotional  excitement  and  physical  over-exertion. 
Especially  injurious  seems  to  be  the  accumulated  emotional  excitement  of 
the  newly  married.  Within  a  short  time,  I  have  seen  serious  symptoms 
appear  in  six  young  brides  whose  anamnesis  proved  that  the  beginning 
of  the  disease  dated  far  back  into  their  girlhood. 

With  remissions  and  exacerbations  the  disease  gradually  grows  worse. 
The  duration  is  quite  long,  amounting  to  5  to  10  years,  and  even  longer. 

Death  results  from  intercurrent  affections,  or  because  of  the  bulbar 
changes,  occasionally  during  an  apoplectic  attack. 

One  form  of  the  disease  terminates  in  death  very  quickly,  at  most  within 
a  few  months,  or  a  year:  then  one  speaks  of  acute  multiple  sclerosis.  Mar- 
burg, Schlagenhaufer,  Gudden,  Flatau  and  others  have  especially  studied  this 
form.  The  development  of  some  of  these  cases  from  an  inflammatory  proc- 
ess, encephalomyelitis,  is  proved  with  considerable  certainty  by  several 
observers.  I  myself  have  observed  such  a  case  clinically  and  anatomically. 
Oppenheim  has  repeatedly  seen  the  development  of  a  disseminated  sclerosis 
from  an  infectious  myelo-encephalitis. 

Differential  Diagnosis. — Many  affections  are  to  be  considered  in  making 
a  differential  diagnosis.  Of  especial  importance  is  the  differentiation  from 
hysteria.  For  a  number  of  years,  I  have  been  emphasizing  in  my  lectures, 
again  and  again,  the  frequency  of  these  confusions;  Redlich  and  Oppenheim 
have  had  the  same  experience.  In  every  case  of  hysteria  with  ankle-clonus 
the  possibility  of  a  disseminated  sclerosis  must  be  considered;  furthermore, 
also  in  every  case  of  hysteric  amaurosis,  in  quickly  disappearing  disturb- 
ances in  gait  and  pareses  in  hysterical  individuals,  should  signs  of  an  affec- 
tion of  the  pyramidal  tracts  be  present.  If  the  Babinski  phenomenon  is 
positive  with  dorsal  flexion  of  the  great  toe,  likewise  Oppenheim' 's  leg  phenom- 
enon, if  there  be  spastic  paresis  in  the  lower  extremities,  an  organic  affec- 
tion must  be  assumed  in  addition  to  hysteria  and  then  multiple  sclerosis 
stands  in  the  front  rank  of  probability.  This  probability  is  increased  if  the 
patient  attacked  is  a  young  girl  or  a  young  woman.  The  further  course,  in 
particular  transient  bladder  disturbances,  enables  the  physician  to  make  a 
diagnosis. 

Differentiation  from  encephalitis  may  become  very  difficult,  especially 
if  the  symptoms  of  an  acute  pontile  or  medullary  affection  appear  in  young 
persons  without  affection  of  the  vessels  or  heart  and  there  is  no  kidney 
disease  or  syphilis.  Detection  of  an  elevation  of  temperature  from  a  directly 
preceding  infectious  disease  would  speak  rather  in  behalf  of  the  diagnosis 
of  an  encephalo-myelitis. 

If  in  a  young  person,  the  acute  lesion  (in  the  form  of  an  apoplectic  attack) 


348  DISEASES  OF  THE  SPINAL  CORD 

affects  the  brain,  the  further  course  of  the  disease  must  be  awaited  if  the 
other  changes  discussed  above  are  absent.  If  the  symptoms  disappear,  and 
absence  of  fever  and  a  directly  preceding  serological  factor  is  noted,  a  pro- 
gressive paralysis  or  multiple  sclerosis  is  probable.  If  the  intellect  is  not 
seriously  disturbed  by  the  attack,  disseminated  sclerosis  is  the  more  probable 
diagnosis. 

But  if  a  lesion  in  the  vessels  (atheroma)  is  present,  even  with  multiple 
foci,  the  decision  must  be  in  favor  of  assuming  the  presence  of  a  softening, 
since  multiple  sclerosis  is  more  apt  to  be  found  in  young  people. 

Cerebrospinal  syphilis  can  give  rise  to  a  disease  picture  very  similar  to 
that  of  multiple  sclerosis.  Besides  the  anamnesis,  the  effect  of  specific 
treatment  will  be  of  differential  diagnostic  value  (though  such  treatment 
must  be  used  with  caution). 

Cases  of  solitary  as  well  as  multiple  tumors  of  the  brain  have  been  ob- 
served, which  clinically  bore  a  close  resemblance  to  disseminated  sclerosis 
(Bruns,  Nonne);  in  the  latter  even  choked  disc  has  been  observed.  Of 
course  this  is  not  a  common  occurrence.  The  absence  of  disturbed  intellect 
and  of  constant  headache  militates  against  a  diagnosis  of  a  tumor,  whereas 
signs  of  constantly  increasing  intra-cranial  pressure  make  the  existence  of 
a  tumor  more  probable. 

Many  years  ago,  Charcot  separated  multiple  sclerosis  and  paralysis 
agitans  diagnostically  in  their  clinical  phenomena.  Paralysis  agitans  is  a 
disease  occurring  in  older  persons;  the  tremor  of  shaking  palsy  is  unlike 
intention  tremor;  nystagmus  and  scanning  speech  do  not  belong  to 
paralysis  agitans,  while  the  general  rigidity  is  not  peculiar  to  multiple 
sclerosis. 

Friedreich's  disease,  or  really  heredo-ataxie  cerebelleuse  (Marie)  which  is 
distinguished  from  it  by  many  authors,  may  closely  resemble  certain  stages 
of  multiple  sclerosis.  In  heredo-ataxia  there  is  nystagmus  and  a  disturb- 
ance in  speech,  which  recalls  scanning  speech;  the  tendon  reflexes  may  be 
increased.  A  choreiform  tremor  can  affect  almost  the  entire  musculature 
of  the  body  and  become  intensified  upon  motion.  The  gait,  however,  is 
ataxic,  occasionally  a  peculiar  club  foot  develops  and  an  optic  atrophy,  which 
does  not  improve. 

Progressive  paralysis  quite  often  exhibits  a  certain  similarity  to  multiple 
sclerosis.  But  the  stumbling  over  syllables,  the  reflex  pupillary  rigidity, 
the  trembling  speech,  early  psychic  disturbances,  unequal  innervation  of 
the  facial  musculature,  and  the  trembling  of  the  lips  are  peculiar  characteris- 
tics of  the  paralysis  and  not  of  multiple  sclerosis. 

Differentiation  from  the  affection,  which  was  described  by  Westphal  as 
pseudo-sclerosis  and  later  was  specially  studied  by  Striimpell  and  v.  Frankl- 
Hochwart,  may  prove  difficult.  These  are  comparatively  rare  cases,  pre- 
senting many  of  the  clinical  symptoms  of  disseminated  sclerosis,  while  no 


MULTIPLE  INSULAR  SCLEROSIS  349 

anatomic  change  can  be  found  postmortem.  This  affection  is  distinguished 
from  multiple  sclerosis  by  the  absence  of  nystagmus,  of  the  optic  affection 
and  also  of  the  genuine  spastic  phenomena.  The  early  development  and 
the  severe  psychic  disturbances  (dementia)  are  conspicuous;  Strilmpell 
emphasized,  besides,  the  fact  that  the  tremor  appeared  even  during  rest, 
was  slower,  but  of  greater  amplitude.  The  active  movements  also  are  decid- 
edly retarded.  Speech  is  scanning,  but  also  clearly  dysarthric.  Epilepti- 
form and  apoplectiform  attacks  are  present  in  almost  every  case.  On  the 
basis  of  these  phenomena,  correct  diagnoses  were  made  in  vita  by  Strilmpell 
and  v.  Frankl-Hochwart. 

At  present,  pseudo-sclerosis  cannot  be  distinguished  from  genuine  diffuse 
cerebral  sclerosis  with  degeneration  of  the  pyramidal  tracts  (Striimpell, 
Oppenheim) .  The  latter  author  is  also  of  the  opinion  that  the  combination 
of  progressive  spastic  paralysis  with  progressive  dementia  in  childhood 
must  arouse  a  suspicion  of  diffuse  cerebral  sclerosis. 

Therapy. — One  is,  alas,  comparatively  helpless,  face  to  face  with  the 
development  of  the  disease,  and  can  only  sometimes,  guided  by  a  knowledge 
of  the  peculiarities  of  its  course,  obtain  remissions. 

I  have  seen  but  few  benefits  follow  the  use  of  medicines.  With  anaemic 
persons,  especially  in  cases  of  a  low  state  of  nutrition,  preparations  of  iron 
and  arsenic  may  well  be  used.  In  such  cases  I  prefer  ferratin,  ferrum  cacody- 
licum  or  ferrum  lacticum  in  combination  with  preparations  of  quinine,  or 
prescribe  Blaud's , pills;  arsenic  I  prefer  to  administer  subcutaneously,  in- 
tramuscularly, as"  sodium  cacodylate  (every  other  day  0.02  in  a  watery 
solution),  or  as  atoxyl  (every  other  day  0.05-0.10  in  a  watery  solution).  Op- 
penheim has  seen  bad  results  from  mercurial  treatment. 

Of  great  importance  is  the  carrying  out  of  a  strict  rest  cure.  In  poorly 
nourished  patients  synchronous  forced  feeding  is  of  considerable  value. 
The  use  of  (not  prolonged)  lukewarm  baths  or  baths  of  carbonic  acid  (lasting 
from  10  to  15  minutes,  temperature  between  88°  and  940  F.)  seems  to  me 
advantageous  since  they  often  influence  the  rigidity  very  favorably.  If  the 
treatment  by  forced  feeding  and  rest  is  continued  long  enough,  and  the  patient 
takes  very  good  care  of  himself  afterwards,  a  long  remission  may  appear.  At 
present  I  am  treating  a  young  woman,  who  was  paraplegic  four  years  ago, 
had  bladder  trouble  and  had  so  far  recovered  by  means  of  forced  feeding 
and  rest,  that  she  could  manage  the  household  for  years,  until  over-exertion 
and  excitement  paved  the  way  for  a  return  of  the  paraplegia.  Such  long 
remissions  I  have  repeatedly  seen. 

I  have  seen  strikingly  favorable  results  from  treatment  by  baths  in  Bad 
Gastein. 

Massage  seems  to  act  favorably.  Oppenheim  recommends  that  passive 
movements  be  used  in  the  bath. 

Steam  baths,  hot  baths,  affect  unfavorably  the  course  of  the  disease; 


35©  DISEASES  OF  THE  SPINAL  CORD 

deterioration  may  follow  immediately  upon  the  bath.  The  same  holds 
true  of  all  exciting  hydriatic  and  other  procedures. 

Physical  over-exertion  may  induce  rapid  deterioration;  likewise  under- 
nutrition and  psychic  excitement  may  cause  rapid  progress  of  the  disease. 

Strong  spices,  highly  seasoned  food  and  alcoholic  drinks  are  to  be  avoided. 

Sexual  excesses  in  one  of  my  cases,  caused  in  a  short  time  complete 
development  of  the  disease  which  had  been  up  to  then  but  slightly 
troublesome. 

The  abuse  of  tobacco  also  seems  to  be  followed  by  an  advance  in  the 
disease. 

4.  SYRINGOMYELIA,  SPINAL  GLIOSIS 

BY 

HERMANN  SCHLESINGER  (Vienna) 

Pathological  conditions  of  different  aetiology  are  embraced  by  the  term 
syringomyelia.  They  lead  to  the  formation  of  long,  extensive  cavities, 
which  with  special  frequency  occupy  the  central  sections  of  the  spinal  cord. 
They  also  cause  considerable  tumor-like  proliferations  of  the  glia.  We 
are  dealing  altogether  with  progressive  processes;  the  stationary  or  retrogres- 
sive cystic  degenerations  of  the  spinal  cord  are  to  be  separated  from  syrin- 
gomyelia (Kienbock) .  The  clinical  picture  is  very  variable,  but  permits 
common  traits  to  be  recognized  in  the  individual  cases. 

Pathological  Anatomy  and  Pathogenesis 

The  spinal  cord  is  frequently  flattened  out  for  long  stretches,  and  much 
reduced  in  substance;  it  is  true,  it  frequently  appears  swollen  in  smaller 
sections.  If  the  cavity  extends  to  the  medulla  oblongata,  the  latter  becomes 
very  asymmetrical.  Only  in  a  minority  of  cases  does  one  find  a  diffuse  tumor- 
like swelling  of  the  spinal  cord  with  cavity  formation.  In  cross-section,  one 
sees  a  cavity  occupying  the  central  portion  or  even  a  large  part  of  the  dorsal 
portion  of  the  spinal  cord;  the  size  of  the  cavity  varies  greatly  at  levels  but 
little  distant  from  each  other.  Frequently  there  are  no  large  cavities 
at  all  and  instead  one  finds  a  long  extended  tumor,  which  occupies  the 
central  area  of  the  spinal  cord,  appearing  now  like  a  hard  narrow  glia 
tube,  now  presenting  a  decidedly  tumorous  appearance.  Pure  unilateral 
localization  of  the  disease  is  rare;  even  then  the  gray  matter  is  preferably 
attacked  (posterior  horn).  Most  frequently,  the  cervical  portion  of  the  cord, 
the  cervical  enlargement  and  the  upper  dorsal  segment  are  the  seat  of  the 
lesion.  Often,  the  extent,  lengthwise,  is  very  considerable  and  the  changes 
go  far  down  into  the  lumbar  segment,  indeed  even  into  the  sacral  portion  at 
one  end  and  into  the  bulbus  medullas  at  the  other.     Cases  in  which  the  dis- 


MORBID  ANATOMY  OF  SYRINGOMYELIA 


35i 


ease  is  limited  to  the  lowest  sections  of  the  spinal  cord  or  to  the  medulla 
oblongata  alone,  are  of  rare  occurrence. 

Most  frequently  injured  are — the  region  of  the  central  canal,  the  posterior 
horns  and  the  posterior  columns;  the  latter  are  sometimes,  for  long  stretches, 
entirely  swallowed  up  in  the  formation  of  the  tumor  or  cavity,  but  usually 
suffer  extensive  destruction  only  in  their  ventral  parts  (Fig.  76).  The  lateral 
pyramidal  tracts  are,  as  a  rule,  interrupted  at  several  points,  and  show 
descending  degeneration  on  one  or  both  sides.  The  other  parts  of  the  cross- 
section,  as  the  lateral  cerebellar  tracts  and  the  anterior  columns,  are  affected 
less  frequently,  but  at  any  rate  occasionally,  while  the  anterior  horns  are 
affected  quite  frequently.  '  | 

In  the  medulla  oblongata,  the  lesion  very  rarely  (Spiller  describes  such  a 
case)  extends  beyond  the  lower  margin  of  the  pons  in  the  direction  of  the 


Fig.  76. — Syringomyelia.     Cavity  behind  the  central  canal.     {After  Schmaus.) 


cerebrum.  The  changes  occupy,  preferably,  definite  places  of  the  cross- 
section  (H.  Schlesinger) ;  usually  either  lateral  or  medial  fissures  are  present. 
As  a  rule,  the  lemniscus,  the  nuclei  of  the  vagus,  the  accessorius,  glosso- 
pharyngeus,  hypoglossus  are  affected,  often,  also,  the  spinal  root  of  the  fifth 
nerve  is  destroyed  (Fig.  77)  {Schlesinger,  Hatschek,  A.  Westphal,  Philipp- 
Oberthiir). 

The  tumor  is  built  up  of  glia  cells  and  glia  fibres ;  likewise  the  connective- 
tissue  elements  play  an  important  role.  The  cavities  frequently  have  an 
ependymal  lining  for  a  shorter  or  longer  part  of  their  course. 

The  origin  of  syringomyelias  through  the  breaking  down  of  these  tumor- 
like glioses  was  pointed  out  first  by  Simon,  Westphal,  later  by  Schultze, 
Hoffmann,  Oppenheim,  Schlesinger  and  others. 

The  condition  is  termed  a  hydromyelia,  if  the  cavity  is  lined  throughout 
with  ependyma,  a  syringomyelic  gliosis  (Schlesinger),  if  the  cavities  are 
combined  with  gliosis  or  have  originated  in  the  latter.     The  fissures  of  the 


352  DISEASES  OF  THE  SPINAL  CORD 

spinal  cord  are  surrounded  by  indurated  masses  of  glia  connective  tissue, 
while  the  syringobulbia  is,  as  a  rule,  bounded  by  degenerating  nerve  tissue. 
In  the  cavities  and  also  in  their  walls  are  often  found  residues  of  hemor- 
rhages and  changes  in  the  vessels.  The  glioses  do  not  extend  to  the  men- 
inges. If  extensive  meningeal  changes  with  cavity  formations  do  exist,  the 
condition  is  not  one  of  glio-syringomyelia. 

As  predisposing  to  the  development  of  syringomyelia,  we  might  consider 
anomalies  in  the  development  of  the  entire  central  nervous  system  or  of  the 
central  canal  (Leyden)  and  its  immediate  vicinity.  Most  likely  glioses 
(tumor-like,  degenerating  proliferations  of  glia)  come  from  a  congenital  or 
early  acquired  disposition  (Hoffmann,  H.  Schlesinger) .  Traumatism  seems 
occasionally  to  lead  to  the  development  of  glioses  {Minor,  Westphal),  but  a 
certain  predisposition  is  probably  essential. 


Fig.  77. — Syringobulbia  with  degeneration  of  the  lemniscus.     {After  Schmaus.) 

F.  Schultze  has  made  it  appear  probable  that  traumatisms  during  birth 
form  the  starting  point  of  the  affection.  In  compression  of  the  spinal  cord, 
a  syringomyelia  is  found  occasionally  above  the  place  of  compression. 
Infectious  diseases  bring  a  gliosis  in  their  train  relatively  often.  Syphilis 
(Schwarz,  Simon)  and  meningitis  may  be  followed  by  syringomyelia;  possibly 
meningeal  and  spinal  affections  are  co-ordinated  (Midler,  Meder  and  others) . 
Changes  in  the  vessels  can  also,  especially  in  advanced  age,  cause  formation 
of  fissures  (without  gliosis).  The  aetiological  role  of  inflammatory  spinal 
processes,  for  instance,  myelitis  (Joffroy,  Hallopeau),  for  the  origin  of  syringo- 
myelia is  still  debatable. 

For  syringobulbia,  changes  in  the  vessels  are  of  importance,  as  well  as 
anomalies  in.  development. 

The  anatomic  aetiology  of  syringomyelia  is  therefore  not  uniform. 

Besides  infections,  intoxications  might  also  be  an  aetiological  factor  here 
and  there.  But  this  influence  is  occasionally  exaggerated.  For  the  pro- 
duction of  a  syringomyelia  by  peripheral  traumata  there  is,  as  yet,  no  proof. 


SYRINGOMYELIA 


353 


Symptoms. — The  clinical  picture  of  syringomyelia  is  extraordinarily 
varied.  The  phenomena  that  come  under  observation  belong  preponder- 
antly to  the  following  groups  of  symptoms :  i.  Phenomena  in  the  domain 
of  the  motor  nerves;  2.  disturbances  in  sensation;  3.  vasomotor  trophic 
disturbances;  4.  bulbar  symptoms. 

The  motor  disturbances  are  mainly  muscular  atrophies  in  the  upper  and 
frequently,  also,  spastic  conditions  in  the  lower  extremities. 

The  muscular  atrophy  begins  most  frequently  insidiously  in  the  mus- 
culature of  the  hand.  No  other  spinal  affection  leads  so  frequently  in  young 
persons  to  atrophy  of  the  smaller  muscles  of  the  hand.  Since  the  muscles 
do  not  always  become  atrophied  in  the  same  order,  various  positions  of  the 
hand  can  result.     The  interosseous  spaces  frequently  sink  in,  the  fingers 


Fig.  78. — Cheiromegaly  in  syringomyelia  (as  compared  with  the  normal  hand). 

are  over-extended  at  the  metacarpo-phalangeal  joints,  flexed  at  the  inter- 
phalangeal  joints,  the  tendons  of  the  flexors  become  prominent  in  the  hollow 
of  the  hand,  thenar  and  antithenar  muscles  become  atrophied,  so  that  the 
position  known  as  " claw-hand"  results,  or  through  atrophy  of  the  ball  of 
the  thumb  and  the  antithenar  eminence,  with  a  change  in  the  position  of 
the  thumb,  there  develops  the  "ape's  hand."  Or  through  early  paralysis 
of  the  flexors  of  the  wrist-joint,  the  dorsal  musculature  of  the  forearm 
becomes  the  stronger,  thus  causing  the  development  of  the  so-called 
"preacher's  hand." 

In  many  cases,  however,  the  muscular  atrophy  begins  in  the  muscles 
about  the  shoulder-girdle  and  extends  to  the  musculature  of  the  arm  or  of 
the  hand  only  after  a  longer  time.     Scapular  "angel's  wings"    are  very 
23 


354  DISEASES  OF  THE  SPINAL  CORD 

frequent  in  these  cases  since  the  muscles,  that  hold  the  scapula  in  its  position, 
become  atrophied.  The  cucullaris  is  frequently  atrophied  only  in  its- 
lower  portion. 

It  is  much  less  common  for  atrophy  to  appear  early  in  the  lower  extrem- 
ities; it  can  then  lead  to  the  formation  of  a  pes  equinus.  The  atrophies  are 
mostly  asymmetrical. 

In  the  atrophic  muscles  fibrillary  twitching  is  commonly  observed. 
Reactions  of  degeneration  cannot  always  be  demonstrated. 

Rigidity  is  often  present  in  one  or  both  of  the  lower  extremities.  The 
rigidity  can  spread  to  the  larger  part  of  the  musculature  of  the  body,  so  that 
the  patients  find  themselves  unable  to  move  (Raymond,  Dej  trine,  Redlich). 

The  tendon  reflexes  in  the  lower  extremities  are  increased.  Ankle 
clonus  is  regularly  present.  In  the  areas  of  the  muscular  atrophies,  the 
tendon  reflexes  are  usually  diminished. 

Oppenheim's  leg  phenomenon  and  the  Babinski  toe  reflex  (dorsal  flexion 
of  the  great  toe)  are  present. 

Spontaneous  movements  are  observable  in  all  stages  of  the  disease, 
especially  in  the  fingers  and  thumbs  (Schwarz). 

In  muscles,  not  wholly  able  to  perform  their  functions,  there  occasionally 
appear  symptoms  peculiar  to  myotonia,  a  condition  that  I  designated  as 
myotonia  syringomyelica  (observed  by  Rybalkin,  Fuchs  and  others). 

The  disturbance  in  sensibility  is  only  partial  in  the  great  majority  of 
cases.  As  a  rule,  analgesia  and  thermo-anaesthesia  are  present,  while  the 
other  sensations  suffer  qualitatively  none  or  but  slight  disturbances  ("  syringo- 
myelic dissociation  of  sensation,"  first  established  by  Kahler  and  F.  Schultze). 
The  partial  paralysis  of  sensation  is  demonstrable  over  a  larger  or  smaller 
area  of  the  surface  of  the  body.  It  shows  as  a  rule  (Laehr,  Hahn,  Dtjerine, 
v.  Solder,  H.  Schlesinger  and  others),  segmental  arrangement  and  also  an 
extension  corresponding  to  the  segments  of  the  spinal  cord.  The  area  of 
its  extension  does  not  necessarily  correspond  with  that  of  the  localization  of 
the  muscular  atrophies.  The  disturbances  in  sensibility  may  also  affect 
the  pain  sense  alone,  even  the  sensation  for  cold  and  heat  alone. 

The  defects  in  sensibility  are  also  asymmetrical;  a  sensory  hemiplegia 
has  been  repeatedly  observed. 

The  thermo-anaesthesia  leads  to  the  burns  that  are  so  frequent  in  syringo- 
myelia; they  occur  in  typical  places  (hands,  fingers,  shoulder  blade,  buttocks). 
Tactile  sensibility  and  the  muscular  sense  are  not  infrequently  disturbed, 
but  the  syringomyelic  dissociation  dominates. 

In  the  initial  stage,  sensory  irritation' phenomena  (paresthesias  affecting 
the  temperature  sense)  are  usually  present. 

The  sensibility  of  the  bones  is  often  considerably  lowered  (Egger, 
Neutra) . 

The  trophic  disturbances  affect  the  skin,  bones  and  joints. 


SYMPTOMATOLOGY  OF  SYRINGOMYELIA  355 

The  affections  of  the  skin  are  so  manifold,  that  practically  the  entire 
subject  matter  of  dermatology  would  have  to  be  discussed,  if  one  wished  to 
enter  upon  this  phase. 

The  urticaria-like  eruptions  (dermographism),  the  formation  of  bullae 
which  always  return  in  the  same  places  on  the  body,  are  of  especial  impor- 
tance. Decided  thickenings  (up  to  one  centimeter)  in  the  skin  of  the  palm 
of  the  hand  approach  to  the  forms  of  Morvan's  type.  Deep  rhagades  with 
but  slight  tendency  to  heal,  complicate  this  change.  Vitiligo,  sclerodermic 
changes,  discolorations  of  the  skin,  especially  in  the  hands,  are  common. 
Peculiar  is  the  "main  succulente"  described  by  Marinesco,  in  which  through 
a  transformation  of  the  tissue  of  the  subcutis,  the  skin  has  a  pasty  appearance, 
the  atrophies  being  masked.  But  there  is  actually  no  edema  of  the  skin. 
The  change  takes  place  also  in  the  skin  of  the  fingers.  Very  frequently 
there  develop  in  the  fingers  painless  whitlows,  which,  if  repeated,  cause 
extraordinary  malformations  of  the  fingers  and  the  hand.  Not  rarely,  in 
these  painless  inflammatory  processes,  phalanges  are  lost.  The  nails  become 
misshapen,  claw-like,  rudimentary.  Perforating  ulcer  of  the  sole  of  the  foot 
is  rarer  and  must  always  remind  one  of  a  process  which  clinically  closely 
resembles  syringomyelia — namely,  leprosy. 

Extensive  spontaneous  gangrenes  occur,  but  are  not  peculiar  to  syringo- 
myelia. 

The  disturbances  in  perspiration  are  very  interesting.  The  secretion  of 
sweat  is  increased,  or  anidrosis  develops.  I  also  have  described  "paradoxic 
sweat  secretion,"  perspiring  under  the  influence  of  cold.  The  anomalies  of 
perspiration  affect  only  individual  sections  of  the  surface  of  the  body,  the 
demarcation  of  which  is  parallel  to  the  spread  of  segmental  disturbances  in 
sensibility. 

The  most  striking  affections  of  the  bones  are  spontaneous  fractures,  which 
occur  preferably  in  the  lower  extremities;  their  whole  course  is  unattended  by 
pain  and  they  result  mostly  from  slight  traumatism.  Union  often  takes 
place  slowly  with  formation  of  a  large  amount  of  callus.  The  bones  of  the 
forearm  are  preferred. 

The  fracture  is  remarkably  often  a  pure  transverse  fracture  (Gnesda). 
Tedesko  found  in  the  systematic  X-ray  testing  for  transparency  in  the  bones 
of  syringomyelic  patients  many  cases  with  changed  structure. 

Spontaneous  bone  necroses  of  varying  extent  have  been  described  several 
times,  likewise  formation  of  multiple  exostoses. 

Scoliosis  is  often  present,  possibly  in  a  quarter  of  the  cases  (Bernhardt) ; 
it  can  attain  high  degrees,  is  combined  with  kyphosis  and  predominant  in  the 
thoracic  spinal  column.  The  kypho-scoliosis  usually  develops  slowly,  but 
may  be  an  early  symptom  (Charcot,  Oppenheim,  Roth). 

The  chest  is  often  deformed,  a  trough-like  sinking  in  at  the  upper  sternal 


356  DISEASES  OF  THE  SPINAL  CORD 

end  and  the  neighboring  ribs  ("Thorax  en  bateau" — Marie,  Astie)  is  con- 
sidered characteristic  for  syringomyelia. 

In  the  course  of  the  disease,  a  hand  occasionally  grows  to  gigantic  size, 
but  not  regularly;  cheiromegaly  (Hofmann,  Marie)  (Fig.  78).  The  partial 
gigantic  growth  may  also  affect  one  foot — podomegaly  (H.  Schlesinger). 
The  typical  picture  of  acromegaly  is,  however,  not  simulated  by  these 
conditions. 

•  (  Bladder  and  rectal  disturbances  belong  to  the  early  symptoms  only  in  the 
lumbo-sacral  forms  of  syringomyelia;  otherwise  they  develop,  if  at  all,  only 
in  the  later  stages  of  the  disease.  Sphincteric  spasm,  afterwards  incontinen- 
tia urinae  are  the  most  frequent  anomalies.  The  cystitis  may  run  a  wholly 
painless  course. 

Diabetes  insipidus,  glycosuria,  were  observed  in  a  number  of  cases.  I 
have  seen  nephrolithiasis  in  several  cases. 

Disturbances  in  the  realm  of  the  sexual  sphere  are  unusual. 
Hemiatrophia  faciei  has  been  observed  as  a  striking  complication  in 
several  cases. 

Bulbar  lesions  are  common  clinically  as  well  as  anatomically.  They  are 
either  unilateral  or  asymmetric. 

The  tongue  is  often  affected.  Then  there  develops  a  hemiatrophy  with 
wrinkling  of  the  mucous  membrane,  fibrillary  twitchings  and  considerable 
diminution  in  size  of  one-half  of  the  tongue  (Lamacq,  Maixner,  Hitzig, 
Raymond,  Schlesinger) . 

Hemiatrophia  linguse  is  often  combined  with  unilateral  paralysis  of  the 
musculature  of  the  palate  and  the  larynx,  so  that  the  symptom-complex  of 
unilateral  bulbar  paralysis  is  present. 

The  disturbances  in  deglutition  are  occasionally  considerable:  faulty 
swallowing,  regurgitation  of  liquids  through  the  nose,  remaining  of  bits  of 
food  upon  the  base  of  the  tongue.  They  may  be  transitory. 

The  laryngeal  paralyses  are  characterized  by  a  total  palsy  of  the  recurrent 
laryngeal  nerve  with  simultaneous  paresis  of  the  soft  palate  and  of  the 
muscles  of  deglutition  (H.  Schlesinger,  Baurovicz) .  Atrophy  of  a  vocal  cord 
is  common;  the  external  branch  of  the  recurrens  need  not  be  injured. 

Paralyses  in  taste  occur,   sometimes  in  individual  qualities  of  taste. 
Speech  may  be  decidedly  bulbar  or  hoarse  with  slight  change  of  voice 
(consequence  of  the  recurrens  paralysis). 

Often  the  bulbar  symptoms  appear  with  an  intense  attack  of  vertigo  with- 
out loss  of  consciousness.  The  feeling  of  dizziness  is  of  extraordinary  vio- 
lence, so  that  the  patients  fall  to  the  ground.  Immediately  after  this  feeling 
of  vertigo,  the  bulbar  paralysis  appears,  or  the  symptoms  of  it  already 
present  become  more  progressive.  ' 

In  many  cases,  a  unilateral  lesion  of  the  sensory  trigeminus  is  the  first 
symptom  of  bulbar  disease  (the  spinal  trigeminal  root  is  attacked).     The 


SYMPTOMATOLOGY  OF  SYRINGOMYELIA 


357 


extension  of  the  disturbances  in  sensation  in  the  scalp,  and  in  the  face  follows 
with  sharp  boundaries  that  are  not  to  be  found  in  peripheral  trigeminus 
lesion.  The  disturbance  in  sensation,  which  in  most  of  the  cases  is  partial, 
is  bounded,  as  it  advances,  by  lines  which  are  packed  closely  into  each 
other,  in  the  form  of  an  onion,  about  the  mouth  or  the  nose,  and  which  have 


Fig.  79. — Paralysis  of  the  sympathetic  at  the  left  side  in  a  case  of  unilaterally  localized 
syringomyelia  (gliosis  unilateralis) .     (After  Heinr.  Curschmann.) 

been  closely  studied  by  v.  Solder  and  myself.  This  type  of  disturbance  in 
sensibility  corresponds  to  the  segmental  arrangement  of  the  elemental  con- 
stituents of  the  trigeminus. 

First,  in  regular  order,  the  laterally  situated  parts  of  the  skin  of  the  face, 
and  only  towards  the  end,  the  most  medially  situated  sections  become  anaes- 
thetic, or  only  analgetic  and  thermo -anaesthetic. 


358  DISEASES  OF  THE  SPINAL  CORD 

Neuralgia-like  pains  in  the  face  are  present,  especially  in  the  beginning  of 
the  bulbar  affection. 

Trophic  disturbances  (corneal  affections)-  which  often  accompany  a  tri- 
geminus lesion,  are  rare. 

Facial  paralyses  are  rarer,  may  strike  now  only  the  buccal  branch,  now 
the  entire  facialis.  The  paralysis  is  located  on  the  side  of  the  more  pro- 
nounced defects  in  sensibility  in  the  trunk. 

The  ocular  disturbances  are  of  various  kinds.  Very  common  is  nystagmus 
or  nystagmus-like  twitchings  in  turning  the  eyes  to  either  side.  Of  paralysis 
of  the  muscles  of  the  eye,  the  abducens  paralyses  are  most  frequent;  the 
Argyll-Robertson  phenomenon,  which  has  been  observed  several  times,  is,  as 
a  rule,  to  be  traced  back  to  a  complicating  disease  (tabes  or  progressive 
paralysis) . 

Very  frequently,  in  about  one-sixth  of  the  cases,  a  paralysis  of  the  sympa- 
thetic with  all  its  typical  phenomena  (sunken  eyeball,  moderate  degree  of 
ptosis,  contraction  of  pupils,  which  retain  the  ability  to  react)  is  present 
(Fig.  79).  Besides,  there  are  vasomotor  phenomena  in  the  same  half  of 
the  face  and  unilateral  disturbances  in  the  secretion  of  sweat  (anidrosis  or 
hyperidrosis) . 

The  visual  field,  in  the  great  majority  of  cases,  where  there  is  no  com- 
plication with  hysteria,  is  normal.  Neuritis  and  choked  disc  have  been 
described  several  times. 

The  bulbar  disturbances,  therefore,  affect  mainly  the  fifth  to  the  twelfth 
cerebral  nerves. 

Common  Clinical  Types. — Very  frequently  one  sees  a  syringomyelia 
with  the  "classic  symptoms"  when  the  disease  is  confined  to  the  cervical 
segment  of  the  cord.  Then  there  are  present,  usually,  muscular  atrophy  in 
the  hands  of  the  Aran-Duchenne  type,  dissociated  disturbances  in  sensibility 
and  trophic-secretory  disturbances  in  the  upper  half  of  the  trunk  and  the 
upper  extremities.  Or  the  muscular  atrophy  begins  in  the  musculature  of 
the  shoulder-girdle  and  is  accompanied  in  earlier  or  later  stages  by  disturb- 
ances in  sensation  (humero-scapula  type). 

Often  in  the  early  stages  of  the  disease,  the  disturbances  in  sensibility 
are  but  slightly  developed  and  their  existence  demonstrated  with  difficulty. 

Symptoms  of  spastic  spinal  paralysis,  or  of  an  amyotrophic  lateral 
sclerosis  are  not  very  rare.  In  both  cases  disturbances  in  sensation  may 
long  be  absent  or  be  only  slightly  developed,  and  the  diagnosis  will  have  to 
be  made  from  trophic  disturbances  of  the  skin,  the  bones,  or  the  joints. 

In  the  lower  extremities  also,  onset  with  the  well-known  triad  of  symp- 
toms is  possible.  Bladder  disturbances  are  more  common  in  these  forms 
than  in  others,  just  as  is  perforating  ulcer  of  the  foot.  The  syringomyelic 
pes  varo-equinus  is  usually  found  with  this  form  (lumbosacral  syringomyelia) . 

I  have  already  mentioned  the  syringomyelia,  that  is  confined  to  the 


TYPES  OF  SYRINGOMYELIA 


359 


lowest  segments  of  the  spinal  cord  {sacral  form) .  In  one  case  observed  by 
me,  there  were  constantly  present  recurring  perforating  ulcers  in  both  heels, 
disturbances  in  the  sexual   sphere,  loss  of  libido  and  ability  of  erection, 


Fig.  80. — Syringomyelia  of  the  Morvan  type.     {After  Heinr.  Curschmann.) 

partial  paralysis  of  sensation  in  the  perineum  and  about  the  anus,  loss  of 
the  feeling  of  a  full  bladder  and  loss  of  the  Achilles  tendon  reflex. 

Beginning  of  the  disease  in  the  medulla  oblongata,  and  a  later  affection  of 
the  medulla  spinalis  is  far  less  frequent  than  secondary  syringobulbia. 

Sometimes  the  gliosis  attacks  a  posterior  horn  to  a  considerable  extent 


360  DISEASES  OF  THE  SPINAL  CORD 

lengthwise  (Rossolimo,  Oppenheim) ;  then  there  occur  unilateral  disturbances 
in  sensibility,  which  remind  one  very  much  of  hysteric  disturbance.  Only 
in  rare  isolated  cases  has  a  general  anaesthesia  been  observed  in  syringomyelia. 

Through  the  predominance  of  trophic  phenomena,  peculiar  clinical  forms 
are  created,  as  the  osteo -arthritic  form  due  to  early  and  extensive  changes  in 
bones  and  joints.  Cases  with  serious  malformations  of  the  fingers  and 
hands,  as  a  result  of  repeated  whitlows  and  phlegmonous  processes  are 
particularly  frequently  observed.  These  forms  with  unsightly  thickenings 
and  distortions  of  distal  parts  of  the  extremities  and  severe  disturbance  of 
tactile  sensibility  are  designated  as  Morvan's  symptom  complex  (Fig.  80). 

The  tabetic  type  may  originate  from  a  combination  of  tabes  and  syringo- 
myelia (for  instance  tabetic  phenomena  in  the  legs,  syringomyelic  in  the 
arms)  or  by  localization  of  a  gliosis  in  the  posterior  columns. 

Besides  these  types,  there  are  rarer  forms,  caused  by  peculiar  arrange- 
ments of  the  gliosis  or  by  combination  with  other  diseases,  as  gliosis  cruciata 
{Oppenheim)  with  typical  disturbances  in  the  upper  and  lower  limbs  of  oppo- 
site sides.  The  pachymeningitic  forms  soon  lead  to  the  phenomena  of 
transverse  section  of  the  cord  with  motor  and  sensory  paralysis  of  the  lower 
half  of  the  body. 

Combination  with  hydrocephalus  is  not  unusual  and  has  been  observed 
several  times  in  association  with  disturbances  in  the  development  of  the 
bones  and  the  soft  parts. 

Oppenheim  and  Marburg  have  repeatedly  seen  "cervical  ribs"  combined 
with  syringomyelia.  Syringomyelia  has  repeatedly  been  observed  (I  have 
seen  it)  with  cerebral  tumors. 

The  very  common  combination  with  hysteria  is  clinically  important,  as  it 
modifies  many  of  the  phenomena  of  syringomyelia. 

Onset,  Age,  Course. — The  disease,  as  a  rule,  begins  stealthily, exhibiting 
a  preference  for  young  persons.  I  have  several  times  been  able  to  recognize 
it  clinically  in  young  children.  An  onset  in  very  old  age  or  even  in  a  moder- 
ately advanced  age  is  less  frequent,  though  the  presence  of  the  disease  even  in 
old  age  is  not  rare.  It  is  usual,  however,  to  find  that  the  phenomena  began 
a  long  time  before.  The  disease  not  infrequently  lasts  from  20  to  40  years. 
If  the  disturbances  are  not  serious,  it  is  usually  the  injuries  that  drive  the 
patient  to  the  physician.  That  is  why  the  surgeon  is  so  frequently  the 
first  to  see  the  patient.  It  is  comprehensible  that  some  vocations  lead  to 
an  earlier  discovery  of  the  disease  (thus  cooks  and  stokers  seek  a  doctor 
because  of  their  frequent  burns) . 

Syringomyelia  is  found  everywhere,  provided  that  its  variable  clinical 
picture  is  known.  In  my  opinion  it  stands  third  or  possibly  fourth  among 
all  the  diseases  of  the  spinal  cord  with  reference  to  the  frequency  of  its 
occurrence. 

The  affection  has,  it  is  true,  a  general  progressive  character,  but  long 


DIFFERENTIAL  DIAGNOSIS  OF  SYRINGOMYELIA  361 

stationary  periods,  lasting  even  for  many  years  are  seen  in  many  cases. 
Very  long  remissions  have  been  repeatedly  described  (Hatschek,  Bruce,  and 
others).  Acute  exacerbations  are  frequent,  being  observed  especially  with 
localization  of  the  disease  in  the  medulla  oblongata  and  may  possibly  be 
the  result  of  hemorrhages,  softenings  or  edema. 

Complications  may  cause  death.  Among  them  cystitis  with  ascending 
inflammation  is  specially  important,  as  well  as  purulent  infections  of  the 
subcutaneous  connective  tissue  and  of  the  joints.  I  have  frequently  seen 
nephrolithiasis  with  syringomyelia. 

In  spite  of  the  frequency  of  syringobulbia,  the  fatal  outcome  is  but 
very  rarely  caused  by  bulbar  disturbances.  Intercurrent  diseases,  especially 
tuberculosis  and  typhoid,  add  a  large  contingent  to  the  number  of  fatal  cases. 

Differential  diagnosis  must  take  into  consideration  an  extraordinarily 
large  number  of  diseases.  Among  these  the  most  important  practically 
is  leprosy.  A  very  large  number  of  monographs  are  concerned  with  this 
theme,  which  is  of  immense  importance  for  the  question  of  the  spread  of 
leprosy  in  civilized  countries. 

Among  the  most  important  we  may  mention  those  of  Laehr,  Zambaco, 
F.  Schultze,  v.  During,  Lie,  Bergmann,  Jeanselme  and  H.  Schlesinger. 

The  diagnostic  difficulties  are  increased  by  the  fact  that  the  combination 
of  leprosy  with  syringomyelia  (Gerber-Matzenauer)  has  been  observed  with 
certainty.  It  is  true  that  such  a  connection  was  formerly  assumed  several 
times,  even  postulated  as  causal  (Zambaco,  Marestang),  but  this  assertion 
has  not  been  proven.  It  was  supported  by  the  clinical  similarity  which 
especially  Morvan's  type  of  syringomyelia  exhibits  to  a  form  of  leprosy, 
but  in  leprosy,  a  spinal  gliosis  is  absent  from  the  anatomic  phenomena  and 
in  syringomyelia,  the  bacilli  of  leprosy  are  absent. 

According  to  the  present  status  of  the  question,  the  following  factors 
are  to  be  considered:  the  origin  is  now  less  decisive  since  new  leprous  foci 
are  continually  being  discovered;  at  any  rate,  a  case  originating  from  a 
region  which  has  always  been  free  of  leprosy,  speaks  against  this  disease. 
Nerve  thickenings,  especially  the  spindle-shaped,  are  peculiar  to  leprosy;  in 
excised  pieces  the  presence  of  leprosy  bacilli  may  occasionally  be  demon- 
strated. They  are  early  and  regularly  found  in  the  N.  auricularis  magnus. 
Oval  cicatricial  patches  of  the  skin  devoid  of  pigment  with  anaesthesia, 
which  have  come  from  pemphigus  bullae,  point  to  leprosy.  Tubercular 
affections  of  the  skin,  which  are  spread  over  a  large  part  of  the  surface  of 
the  body  and  also  attack  the  face,  are  to  be  similarly  interpreted,  likewise 
attacks  of  fever,  especially  during  progression  of  such  eruptions.  A  bacterio- 
logical examination  of  the  nasal  secretions  may  often  early  reveal  the  specific 
bacillus  in  leprosy  (G.  Sticker) .  Peripheral  facial  paralysis  is  rare  in  syringo- . 
myelia,  frequent  in  leprosy,  especially  with  marked  participation  of  the 
frontal  branch  of  the  facial.     Even  specific  disturbances  of  the  eyes  are  not 


362  DISEASES  OF  THE  SPINAL  CORD 

exactly  rare  in  the  latter  affection.  For  gliosis  the  following  speak:  bladder 
and  rectal  disturbances,  increase  of  the  tendon  reflexes,  spastic  phenomena 
in  the  lower  extremities,  severe  multiple  painless  joint  affections,  the  humero- 
scapular  type  of  muscular  atrophy,  nystagmus,  attacks  of  vertigo,  bulbar 
symptoms,  disturbances  in  perspiration,  segmental  disturbances  in  sensibility, 
whereas  the  sensory  disturbances  in  leprosy  occur  more  in  discrete  areas. 

Since  syringomyelia  may  present  the  symptoms  of  scleroderma  as  well 
as  of  Raynaud's  disease  and  also  of  pemphigus,  differentiation  will  occasion- 
ally be  difficult,  but  can  usually  be  decided  on  the  basis,  of  concomitant 
symptoms,  since  gliosis  beside  these  symptoms,  exhibits  others  more  peculiar 
to  itself  (for  instance  spastic  symptoms,  partial  paralysis  of  sensation, 
bulbar  symptoms,  etc.),  in  short,  symptoms  that  do  not  appear  in  skin 
affections. 

Arthritis  deformans  is  distinguished  from  atypical  forms  of  central 
gliosis  by  the  absence  of  analgesia,  and  partial  macrosomia  by  the  absence 
of  disturbances  in  sensibility. 

Hysteria  is  occasionally  very  difficult  to  distinguish  from  syringomyelia, 
especially  from  the  forms  with  disturbances  in  sensation  without  muscular 
atrophy.  The  bulbar  symptoms,  the  increase  in  the  tendon  reflexes, 
sympathetic  paralysis,  trophic  disturbances  will,  as  a  rule,  make  diagnosis 
easy,  though  hysteria  is  often  combined  with  syringomyelia. 

Root  and  plexus  neuritis  is  distinguished  by  absence  of  the  cord  phenomena 
(reflexes  in  the  lower  extremities  unchanged),  by  the  violent  irritation 
phenomena,  and  the  uniform  disturbance  of  all  qualities  of  sensation. 

Polyneuritis  only  rarely  causes  confusion,  since  its  onset  is  much  more 
quick  and  it  is  usually  bilateral  from  the  beginning. 

A  spondylitis  tuberculosa  can  more  easily  simulate  the  picture  of  a  syringo- 
myelia. The  muscular  atrophies  may  be  similar,  also  the  dissociation  of 
sensation  may  be  analogous  to  that  in  syringomyelia ;  even  the  oculo-pupillary 
symptoms  (sympathetic  paralysis)  occur.  But  the  affection  of  the  bones  is 
recognizable  as  such  by  direct  inspection  or  palpation  or  in  the  radiograph; 
the  spinal  column  is  held  rigidly,  transverse  section  symptoms  caused  by  the 
cord  disease  develop  rather  quickly. 

L  ■*  The  separation  from  hcematomyelia  will  not  be  very  difficult  with  a 
knowledge  of  the  history  of  the  case  (acute  onset,  especially  after  traumatism). 
Furthermore  the  phenomena  in  haematomyelia  have  a  tendency  to  become 
less,  in  syringomyelia  usually  not. 

Syringomyelia  may  be  confused  with  tabes,  since  occasionally  the  clinical 
picture  of  syringomyelia  reminds  one  strongly  of  that  of  tabes.  Tabetic 
disturbances  of  the  cerebral  nerves  (bilateral  posticus  paralysis,  Argyll- 
Robertson's  phenomenon),  loss  of  the  tenderness  to  pressure  in  the  ulnaris, 
paralysis  of  the  muscles  of  the  eye,  and  especially  oculomotor  or  bilateral 
abducens  paralyses  will  suggest  tabes  first,  if  the  other  symptoms  are  specially 


PROGNOSIS  AND  TREATMENT  OF  SYRINGOMYELIA        363 

pronounced  in  the  lower  extremities.  But  if  a  partial  paralysis  of  sensation 
is  permanently  present  in  the  lower  limbs,  if  muscular  atrophies  exist,  if  the 
disturbances  in  the  muscular  sense  are  not  of  a  high  degree,  one  will  have  to 
think  of  syringomyelia,  even  if  there  be  ataxia  and  loss  of  the  patellar  re- 
flexes. Typical  phenomena  in  the  upper  extremities  will  confirm  with 
certainty  the  diagnosis. 

From  the  spinal  amyotrophies  (poliomyelitis  chronica,  progressive  muscu- 
lar atrophy  of  the  Aran-Duchenne  type,  amyotrophic  lateral  sclerosis) 
syringomyelia  can  be  distinguished  by  the  presence  of  dissociated  disturb- 
ances in  sensation,  trophic  disturbances  of  the  bones,  joints  and  skin.  Also 
the  one-sided  and  irregular  bulbar  affection  in  contradistinction  to  the  last 
named  disease,  the  increase  of  the  tendon  reflexes  in  the  lower  extremities  in 
contradistinction  to  the  former  two  diseases,  present  important  signs  in 
regard  to  differential  diagnosis. 

Multiple  insular  sclerosis,  upon  longer  observation,  will  nearly  always  act 
clinically  in  a  different  way  than  syringomyelia.  Especially  the  disturbances 
in  sensation  are  usually  not  permanent;  trophic  disturbances  do  not  play  an 
important  role,  more  extensive  muscular  atrophies  are  quite  unusual. 

Intramedullary  tumors  may  give  rise  to  considerable  diagnostic  difficulties. 
The  following  factors,  among  others,  are  important:  the  course  in  tumor  is 
far  more  rapid  than  in  syringomyelia.  The  Brown-Sequard  symptom  com- 
plex is  a  frequent,  if  only  transitory  occurrence  in  tumors.  The  phenomena 
of  sensory  irritation  are  usually  more  prominent  and  pareses  or  paralyses 
develop  far  more  quickly  than  in  syringomyelia.  Bladder  and  rectal  dis- 
turbances, also,  are  found  early.  Bulbar  disturbances,  if  they  be  present, 
are,  as  a  rule,  a  very  bad  sign,  whereas  in  syringomyelia,  bulbar  symptoms 
may  persist  for  many  years. 

The  prognosis  of  the  disease  is  quoad  vitam  not  unfavorable,  since  even 
when  bulbar  affections  exist,  life  is  usually  prolonged  for  many  years.  Death 
is  caused,  as  a  rule,  by  intercurrent  diseases. 

Treatment  is  more  or  less  powerless  to  cope  with  the  disease.  It  is 
asserted  that  use  of  the  X-rays  on  the  spinal  column,  especially  in  cervical 
syringomyelia,  may  check  the  progress  of  the  disease.  I  have  not  as  yet  seen 
any  such  effect  on  the  progress  of  the  affection. 

Prophylactic  measures  are  important.  The  patients  must  not  expose 
themselves  too  much  to  high  or  low  temperature,  since  there  is  danger  of 
burning  or  freezing.  Severe  physical  work  should  also  be  avoided  as  much  as 
possible,  because  of  a  tendency  in  the  affection  to  the  formation  of  haemato- 
myelias  and  because  of  the  possibility  of  spontaneous  fractures.  The  use  of 
hot  baths  may  bring  about  an  acute  exacerbation  of  the  affection. 

The  muscular  rigidity  is  influenced  favorably  by  protracted  lukewarm 
baths.  The  addition  of  treatment  by  radium  seems  to  me  useful,  likewise 
the  use  of  the  baths  at  Gastein. 


364  DISEASES  OF  THE  SPINAL  CORD 

If  pains  be  present,  the  use  of  the  modern  antipyretics,  especially  of 
pyramidon,  citrophen  or  aspirin,  is  of  service. 

Arthropathies  in  case  of  suppuration  or  of  too  much  effusion  demand 
surgical  treatment. 

5.  ILEMATOMYELIA 

BY 

HERMANN  SCHLESINGER  (Vienna) 

Hemorrhage  into  the  spinal  cord  is  a  much  rarer  affection  than  hemorrhage 
in  the  brain.  We  differentiate  between  primary  and  secondary  hemorrhages 
of  the  spinal  cord,  the  latter  of  which  takes  place  into  a  tissue  that  has  already 
been  changed  (for instance,  tumors,  inflammatory  processes,  etc.).  Hsemato- 
myelia  preferably  affects  the  gray  matter;  the  posterior  horns  are  more  fre- 
quently injured  than  the  anterior  horns.  The  hemorrhage  spreads  length- 
wise in  the  spinal  cord,  may  even  occupy  a  large  part  of  the  medulla  (tubular 
hemorrhage),  may  occupy  one  or  both  sides  of  the  cord,  may  injure  the 
anterior  horn  or  the  posterior  horn  alone,  while  the  lateral  columns  are  usually 
spared  (Minor).  The  localization  and  the  spread  of  the  hemorrhage,  might, 
as  I  assumed  years  ago,  be  dependent  upon  the  loose,  yielding  structure  of 
the  gray  parts  of  the  spinal  cord.  Later  experimental  investigations  by 
Goldscheider,  Flatau  support  this  view.  The  hemorrhages  may  take  place  in 
any  part  of  the  spinal  cord;  they  are  most  frequently  found  in  the  enlarge- 
ments. The  intrameningeal  hemorrhages  are  mostly  of  less  clinical  im- 
portance than  the  intracordal,  as  Thorbum  and  Stolper  have  shown. 

Of  the  ^etiological  factors  traumatism  is  the  most  important  (for  about 
9/10  of  the  cases,  according  to  Oppenheim).  According  to  Stolper,  forced 
flexion  of  the  head  forwards  may  cause  a  hsematomyelia  (for  instance,  diving 
head  foremost) .  In  newly-born  infants  hsematomyelia  was  seen  often  after 
Schultze's  method  of  resuscitation  of  still-born  children  and  difficult  deliveries 
(F.  Schultze,  Litzmanri).  In  children,  after  forced  (Lorenz)  reduction  of 
congenital  dislocation  of  the  hip-joint,  hamiatomyelia  of  the  conus  was  ob- 
served (H.  Schlesinger).  After  forcible  muscular  exertion,  spinal  hemor- 
rhages have  been  observed,  but  a  predisposition  (for  instance,  haemophilia) 
seems  to  be  essential.  In  typhoid,  hsematomyelia  has  been  observed  several 
times,  thus  by  Curschmann,  A.  Schiff.  I  saw  the  disease  associated  with 
morbus  maculosus  Werlhoffii,  other  authors  with  pernicious  anemia,  etc. 
Spontaneous  hsematomyelias  are  exceedingly  rare,  but  undoubtedly  occur. 
One  of  my  patients  developed  it,  while  working  on  a  farm,  without 
traumatism. 

Symptoms. — They  vary  a  great  deal  according  to  the  spinal  level  and 
to  the  extent  of  the  hemorrhage.  Common  to  all  forms  is  the  sudden  onset 
with  the  phenomena  of  an  interruption  in  spinal  conduction.  The  maximum 
of  the  lesion,  i.  e.,  the  greatest  extent  and  intensity  of  the  phenomena  are 


SYMPTOMS  OF  HMMATOMYELIA  365 

reached,  usually,  in  the  first  few  hours  of  the  disease,  far  more  rarely  in  the 
first  few  days.  Part  of  the  symptoms,  which  are  not  at  once  visible  (muscular 
atrophies,  trophic  phenomena  in  the  skin,  etc.),  are  produced  by  the  lesion, 
once  it  is  present,  but  need  a  certain  amount  of  time  for  development.  The 
predilection  (so  striking  anatomically)  for  the  gray  substance  evokes  very 
peculiar  symptom  groupings,  to  which  Minor  especially  has  called  attention. 
Very  often  syringomyelic  dissociation  of  sensibility ,  i.  e.,  analgesia  and  thermo- 
anesthesia, with  other  qualities  of  sensation  retained  or  but  slightly  injured, 
are  demonstrable  in  smaller  or  larger  portions  of  the  surface  of  the  body. 

If  the  lesion  is  in  the  sacral  cord,  bladder  and  rectal  disturbances  and 
occasionally  anomalies  in  the  sexual  sphere,  are  added  to  the  disturbances  of 
cutaneous  sensibility  in  the  perineum  and  about  the  anus. 

With  injury  in  the  lumbar  and  lower  dorsal  segments  of  the  cord,  there 
are  usually  added  also  paralyses  of  the  musculature  of  the  legs  (severe  atro- 
phic paresis  in  one  of  my  cases)  and  the  tendon  reflexes  may  show  important 
disturbances  (loss  or  increase).  Most  frequently,  the  hasmatomyelia  is 
located  in  the  cervical  segment  and  evokes  atrophic  paralysis  of  the  upper 
extremities,  whereas  in  the  lower  extremities,  spastic  paresis  holds  sway. 
The  extent  and  degree  of  muscular  atrophy  vary  extraordinarily.  When 
the  hasmatomyelia  is  deep-seated  (lower  cervical  enlargement)  the  cervical 
sympathetic  of  the  same  side  is  often  affected  (disturbances  in  sweat,  oculo- 
pupillary  phenomena,  vaso-motor  disturbances  in  the  face). 

The  Brown-Sequard  symptom  complex  is  developed  with  remarkable 
frequency.  As  Minor,  Oppenheim  and  others  have  shown,  the  disturbance 
in  sensation  is  partial;  indeed,  it  may  be  for  pain  and  temperature  in  one  leg, 
while  the  other  is  spastically  paralyzed,  and  the  upper  extremity  on  the 
opposite  side  is  more  or  less  atrophic. 

Another  form  corresponds  to  the  "classic"  type  of  syringomyelia;  there 
is  atrophic  paralysis  of  an  upper  extremity  and  partial  disturbance  of 
sensation  in  the  same  extremity. 

Of  irritation  phenomena,  initial  pains  in  the  back  must  be  mentioned, 
which,  however,  are  rarely  overpoweringly  severe. 

Course. — The  disease  attains  its  height,  as  I  have  already  remarked,  in 
the  first  hours  (Kienboeck).  If  serious  complications  exist  (injuries  to  the 
vertebral  column,  lesions  of  internal  organs),  these,  as  a  rule,  cause  death. 
Hsematomyelia,  per  se,  even  when  situated  high,  is  usually  not  fatal.  If 
observation  be  over  a  longer  period  of  time,  one  sees  a  more  or  less  rapid 
retrogression  of  the  symptoms,  which,  occasionally,  is  surprisingly  complete. 
In  one  of  my  cases  (described  by  Labin)  lesion  of  the  cervical  cord,  paraplegia 
of  all  four  extremities  was  present,  and  disappeared  completely;  the  disease 
finally  presented  the  clinical  picture  of  a  conus  lesion.  All  phenomena, 
which  may  be  explained  by  blood  compression,  may  retrogress,  likewise 
those  symptoms  that  can  be  traced  to  an  edema  in  the  substance  of  the 


3 66  DISEASES  OF  THE  SPINAL  CORD 

spinal  cord.  Finally,  those  symptoms  persist  which  are  caused  by  complete 
destruction  of  spinal-cord  substance.  The  permanent  symptoms  are 
disturbances  in  sensibility,  especially  those  of  syringomyelic  character,  since 
they  are  called  forth  by  lesion  of  the  gray  axis  of  the  spinal  cord.  To  these 
are  to  be  added  muscular  atrophies,  which  owe  their  origin  to  destruction  of 
parts  of  the  anterior  horns;  in  the  atrophic  muscles,  the  electric  excitability 
is  changed  (reaction  of  degeneration).  Real  trophic  disturbances  seem  to  be 
exceptional  only,  in  haematomyelia.  The  muscular  atrophies  may  be  very 
slight,  and  are  therefore  easily  overlooked;  this  is  a  condition  of  special 
importance  in  judging  medico-legal  accident  cases  (Oppenheim). 

Diagnosis.  Differential  Diagnosis. — The  diagnosis  is,  as  a  rule,  made 
easily  because  of  the  acute  onset,  the  usually  preceding  traumatism,  and  the 
beginning  retrogression  of  the  phenomena  within  a  short  time  of  the  onset 
of  the  disturbance. 

From  a  differential  diagnostic  standpoint  the  diseases  most  concerned  are 
syringomyelia  (cf.  above),  and  acute  myelitis.  In  the  latter  disease,  I  have 
observed  a  most  acute  onset,  with  paralysis  in  several  hours,  and  death  within 
a  few  days  in  three  cases,  and  the  literature  furnishes  similar  observations. 
In  these  cases,  however,  there  is  usually  fever,  and  the  spontaneous  origin 
also  militates  against  haematomyelia. 

The  greater  prominence  of  irritation  symptoms  (pains,  rigidity  of  the 
spinal  column,  sensitiveness  of  the  muscles  to  pressure),  particularly  when  the 
paralytic  phenomena  are  but  slightly  pronounced,  points  to  hemorrhage  in 
the  meninges  (Haematorrhachis) . 

In  tumors  accompanied  by  hemorrhage  into  the  tumorous  tissue,  the 
anamnesis,  as  a  rule,  points  to  a  longer  duration  of  the  disease. 

In  poliomyelitis,  the  almost  exclusive  anterior  horn  symptoms  and  the 
onset  with  fever,  make  the  diagnosis  possible. 

The  prognosis  of  the  disease  depends,  as  has  been  said  before,  on  the 
complications.     It  is  better,  when  no  bed-sores  and  no  cystitis  develop. 

The  duration  of  the  disease  may  be  considerable.  I  possess  anatomic 
preparations  from  a  case  that  lasted  15  years  (destruction  of  the  sacral  cord). 
I  know  another  case  in  which  haematomyelia  developed  17  years  ago,  and  in 
which  I  have  been  able  to  demonstrate  the  condition  (Conus  lesion)  as 
stationary  for  more  than  10  years. 

Treatment. — The  most  important  therapeutic  factor  is  absolute  rest — 
if  possible,  for  several  weeks.  Oppenheim  recommends  lying  on  the  stomach 
or  side  (marked  tendency  to  bed-sores).  Internally,  gelatin  (2  drams  daily 
in  watery  solution),  calcium  chloride  (20  grains  daily  in  water)  or  adrenalin 
(5  drops  of  the  standard  solution  4  times  a  day)  are  to  be  advised.  Subcu- 
taneous injections  of  ergotin  may  be  beneficial.  Especially  in  meningeal 
conditions  of  irritation,  and  in  full-blooded  persons,  leeches  are  indicated  in 
the  region  of  the  spinal  column. 


FOCAL  DISEASES  OF  THE  SPINAL  CORD  367 

If  atrophic  paralyses  develop,  frequent  lukewarm  baths  may  be  used; 
hot  baths  are  contraindicated. 

Treatment  by  baths  at  Bad  Gastein,  Wildbad,  Nauheim,  Oeynhausen 
are  occasionally  useful  if  the  hemorrhage  has  existed  some  time  (at  least 
5  to  6  weeks).  But  in  treatment  by  baths,  all  the  more  strongly  exciting 
procedures,  and  especially  hot  baths  are  to  be  avoided. 

6.  FOCAL  DISEASES  OF  THE  SPINAL   CORD  DUE  TO  EXTRA- 
AND  INTRA -MEDULLARY  AFFECTIONS 

BY 

R.  FINKELNBURG  (Bonn) 

I.  Traumatic  Diseases  of  the  Spinal  Cord 

(Changes  Due  to  Crushing  (Contusions) ,  Lacerations  and  Concussion) 
Whenever  the  spinal  column  is  injured  either  by  direct  or  indirect  violence, 
that  is,  by  a  blow,  contusion,  by  crushing  or  by  traction  upon  it  or  by  a  fall 
upon  the  head  or  the  buttocks,  the  spinal  cord  may  be  injured  also.  This 
will  be  particularly  the  case  if,  as  a  result  of  fracture  of  the  body  of  a  vertebra, 
or  of  vertebral  dislocation,  there  are  permanent  changes  in  the  position  of  an 
entire  vertebra  or  of  any  of  the  parts  thereof.  The  spinal  cord  may  show 
total  or  partial  contusions  and  lacerations,  root  lesions  with  or  without 
hemorrhages  into  the  membranes.  But  also  after  only  momentary  displace- 
ment of  one  or  more  of  the  vertebral  bodies,  even  if  the  vertebra  has  returned 
to  its  natural  position  upon  relaxation  of  the  force  influencing  it,  severe 
pressure  lesions  of  the  cord  may  be  caused. 

The  seat  of  a  dislocation  and  distortion  is  most  frequently  the  cervical 
portion  of  the  spinal  column ;  the  former  arises  from  forced  flexion,  with  or 
without  simultaneous  rotation  of  the  neck,  the  latter  not  infrequently  from 
violent  stretching  of  the  spinal  column.  In  isolated  vertebral  fractures,  it  is 
a  question  either  of  so-called  compression  fractures,  caused  by  destruction  of 
the  body  of  a  vertebra  by  forces  exerted  upon  its  axis,  or  of  fractures  in  the 
vertebral  arches,  which  but  rarely  give  rise  to  an  injury  of  the  cord  and  the 
roots.  Fractures  in  the  body  of  a  vertebra  occur  mainly  in  the  region  of 
the  lower  dorsal  and  lumbar  vertebrae.  Isolated  lesions  of  the  inter-vertebral 
discs  are  rare;  they  frequently  accompany  dislocations  and  fractures  of  the 
vertebrae. 

Apart  from  the  serious  injuries  of  the  spinal  cord  resulting  from  perma- 
nent or  transitory  compression,  caused  by  vertebral  injuries,  even  after  simple 
concussion  to  the  spinal  column,  coarser  or  finer  spinal-cord  changes  may  arise. 
The  former  consist  in  extra-  or  more  frequently  intra-medullary  hemorrhages 
and  in  softening  of  the  substance  of  the  spinal  cord  with  possibly  additional 
chronic  myelitis  of  the  white  and  gray  matter  at  a  later  period  (cf.  Fig.  81). 


368 


DISEASES  OF  THE  SPINAL  CORD 


Slight  concussional  injuries  in  the  form  of  delicate  changes  in  the  gang- 
lion cells  and  nerve  fibres,  as  may  also  be  evoked  independently  in  experi- 
menting on  animals  by  concussion  upon  the  spinal  column,  frequently  dis- 
appear entirely,  after  leading  at  first  to  serious  phenomena  of  functional  loss. 
But  occasionally  in  connection  with  such  a  commotio  spinalis,  even  if  the  in- 
cipient functional  disturbances  have  disappeared,  there  develop  insidious 

degenerations  of  the  spinal  cord  in  the  form 
of  a  chronic  myelitis.  It  is  of  special 
practical  importance,  that  after  commotio 
spinalis,  in  which  the  brain  also  is  usually 
co-affected,  together  with  organic  changes, 
but  also  very  frequently  without  them, 
pure  functional  disturbances  of  the  nervous 
system  may  appear — traumatic  neurasthe- 
nias and  hysterias.  These  commotion 
neuroses  were  formerly  spoken  of  as  "rail- 
way spine"  since  they  were  observed  es- 
pecially after  railway  collisions. 

Anatomic  Findings. — After  severe 
crushing  there  are  found,  in  addition  to 
hemorrhages  in  the  membranes  and  sub- 
stance of  the  spinal  cord,  marked  flatten- 
ing, diminution,  or  even  complete  divi- 
sion of  the  spinal  cord.  This  is  soon 
followed  by  a  hemorrhagic  softening,  in 
the  further  course  of  which  the  destroyed 
and  liquefied  remnants  of  the  cord  are 

Fig.  81.— Fracture  of  the  first  lumbar-  absorbed  and  partly  taken  up  by  granular 
vertebra  due  to  compression.     {After  Th.  rf>]is 
Kocher.) 

In  the  vicinity  of  the  lesion  there  ap- 
pear inflammatory  reaction  phenomena;  the  glia  cells  and  fibres  swell  and 
multiply;  the  vessels  are  surrounded  by  granular  cells,  and  occasionally 
numerous  round  cells  are  to  be  seen  in  the  area  of  destruction.  The  out- 
come is  a  cicatricial  focus,  consisting  of  glia  tissue  with  thickened  vessels, 
occasionally,  also,  with  the  formation  of  larger  or  smaller  cysts.  Ascend- 
ing and  descending  degenerations  are  added  to  the  picture. 


Phenomena  of  the  Disease 

Immediately  after  every  severe  injury  of  the  spinal  cord,  there  appears, 
during  the  first  effects  of  shock,  the  symptomatic  picture  of  a  complete  inter- 
ruption in  conduction,  even  when  the  spinal  cord  is  but  partly  injured.  It  is 
therefore  only  after  the  first  shock  phenomena  have  passed  away,  that  we  can 


FOCAL  DISEASES  OF  THE  SPINAL  CORD  369 

approach  the  therapeutically  and  prognostically  important  question :  is  there 
a  total  or  only  a  partial  transverse  lesion,  is  there  present  crushing  or  merely 
compression  of  the  spinal  cord? 

1.  Complete  Transverse  Lesion 

The  most  important  sign  of  a  complete  transverse  lesion  is  found  in  the 
complete  paralysis  of  the  musculature  of  the  body  below  the  point  of  the  lesion, 
which  appears  immediately  after  the  injury  and  remains  permanently.  At 
the  same  time  motor  irritation  phenomena,  twitchings,  spasms  in  the  para- 
lyzed region  are  wholly  lacking,  while  they  may  appear  near  the  upper 
boundary.  Even  when  the  seat  of  the  lesion  is  in  the  upper  segments  of 
the  spinal  cord,  the  paralysis  of  the  lower  extremities,  as  a  rule,  is  flaccid, 
and  not  only  during  the  period  immediately  following  the  injury,  but 
usually  afterwards  as  well. 

The  tendon  as  well  as  the  cutaneous  reflexes  are  completely  lost  in  total 
lesions  above  the  lumbar  segment,  not  only  immediately  after  the  injury, 
but  often  permanently.  But  permanent  loss  is  by  no  means  always  the  case. 
The  theory  of  Bastian,  that  every  total  transverse  lesian  of  the  spinal  cord  brings 
with  it  a  permanent  loss  of  the  tendon  reflexes  below  the  point  of  injury,  is 
disproven  by  a  number  of  indisputable  observations,  in  which,  although 
complete  division  of  the  spinal  cord  was  anatomically  demonstrated,  the 
tendon  reflexes  remained  long  after  the  injury  and  occasionally  even  showed 
an  increase. 

The  loss  of  sensation  in  all  its  qualities  is  complete,  up  to  the  level  of  that 
region  of  the  skin  which  is  supplied  by  the  destroyed  segment  of  the  spinal 
cord.  The  sensory  disturbances  extend  only  then  farther  upwards  than  would 
be  expected,  judging  from  the  position  of  the  injured  segment,  if  by  the  crush- 
ing the  root-fibres  originating  in  the  higher  segments  and  passing  down 
through  the  spinal  canal  are  also  destroyed. 

This,  however,  is  by  no  means  always  the  case,  since  in  an  injury,  which 
strikes  roots  and  cord  simultaneously,  the  former  are  frequently  less  injured. 
Sensory  irritation  phenomena  in  the  form  of  radiating  pains,  hyper-  and 
paresthesias  occur  near  the  upper  limit  of  the  disturbance  in  sensation,  never, 
on  the  other  hand,  within  the  sensorily  paralyzed  region. 

Disturbances  of  the  bladder  and  bowels  are  always  present  and  decided. 
There  is,  at  first,  retention  of  urine,  which  later  is  followed  by  a  periodic 
reflex  evacuation  of  the  bladder.     Bed-sores  appear  sooner  or  later. 

2.  Partial  Lesions  of  the  Cord 

In  these,  the  motor  phenomena  of  paralysis  appear,  as  a  rule,  in  the 
foreground  of  the  disease  picture  in  contradistinction  to  the  usually  less  pro- 
nounced sensory  disturbances.     In  lesions,  in  which  only  a  part  of  the 
transverse  section  is  affected,  we  see,  a  short  time  after  the  injury,  the  motor 
24 


370  DISEASES  OF  THE  SPINAL  CORD 

paralysis,  which,  at  first  is  total,  become  transformed  into  one  that  is  incom- 
plete. At  the  same  time,  according  to  Kocher's  observations,  the  parts  of 
the  body  lower  down,  that  is,  especially  the  lower  extremities,  are  more 
severely  affected  by  the  paralysis,  than  the  upper  parts,  like  the  arms.  An- 
other important  sign  of  a  lesion  of  the  cord  that  is  only  partial  is  the  appear- 
ance of  motor  irritation  phenomena,  of  twitchings  and  early  contractures  in  the 
regions  showing  a  motor  paralysis.  If  the  point  of  injury  is  above  the  lumbar 
segment,  there  appears,  as  a  rule,  a  spastic  condition  of  the  musculature  and 
a  pathological  increase  of  the  tendon  reflexes,  also  the  Babinski  phenomenon. 

In  partial  destruction  of  the  cord  sensation  even  in  pronounced  paraplegia 
may  be  but  slightly  disturbed;  furthermore,  the  disturbance  may  at  times  be 
confined  to  definite  qualities  in  sensation  only  and  does  not  extend  nearly 
so  far  upwards  as  the  region  of  the  motor  paralysis.  The  appearance  of 
radiating  pains  and  paresthesias  in  the' paralyzed  limbs  is,  in  a  certain  measure, 
characteristic,  in  contradistinction  to  the  total  transverse  lesions.  If  there  are 
only  disturbances  in  sensation  without  motor  paralysis,  this  indicates  that 
only  a  root  injury  is  present. 

Not  very  infrequently,  the  symptom  complex  of  a  Brown-Sequard  uni- 
lateral lesion  develops  in  injuries  due  to  stabbing,  shooting,  or  crushing: 
spinal  hemiplegia  on  the  side  of  the  injury,  with  later  appearing  spastic  phe- 
nomena, and  considerable  exaggeration  of  the  tendon  reflexes;  on  the  para- 
lyzed side,  there  is  found,  in  addition,  a  hyperesthesia  and  hyperalgesia  of 
the  skin,  and  at  first,  paralysis  of  the  blood-vessels.  In  the  opposite  side  of 
the  body,  which  does  not  present  motor  disturbances,  sensibility  is  impaired, 
with  the  exception  of  the  sense  of  position.  Pain  and  temperature  sensations 
suffer  most  frequently  and  most  severely.  This  typical  picture  of  Brown- 
Sequard  paralysis  is  generally  blurred  because  the  injury  does  not  as  a  rule 
confine  itself  definitely  to  one-half  of  the  spinal  cord,  or,  because  on  account 
of  an  added  traumatic  myelitis,  the  original  semilesion  becomes  transformed 
into  one  diffusely  affecting  the  whole  transverse  area. 

Disturbances  of  the  bladder  and  bowels  are  frequent  concomitants  of  an 
incomplete  transverse  lesion:  but  they  may  be  entirely  absent  in  spite  of 
pronounced  paralytic  phenomena.  In  partial  lesion  of  the  cord,  retention 
of  urine  with  completely  preserved  bladder  sensation  may  be  observed; 
the  sensation  in  many  instances  may  even  be  morbidly  increased,  thus  induc- 
ing violent  vesical  and  rectal  tenesmus,  and  at  the  same  time,  an  impossibility 
of  voluntarily  evacuating  the  bladder  and  bowels.  Priapism  is  no  unusual 
phenomenon  in  partial  lesion;  it  may,  however,  be  lacking  even  in  total 
transverse  lesion. 

For  the  general  ascertainment,  as  to  which  of  the  main  divisions  of  the 
medulla  spinalis  is  affected  by  complete  or  partial  transverse  lesion,  the 
following  table  gives  sufficient  data.  The  exact  determination  of  the  place 
which  is  affected,  in  the  absence  of  external  injuries,  depends  above  all,  on 


FOCAL  DISEASES  OF  THE  SPINAL  CORD 


37i 


the  detection  of  motor  and  sensory  phenomena  of  functional  loss  of  definite 
segments  of  the  spinal  cord.  These  are  best  shown  in  the  tables  of  Edinger 
and  Starr-Brims,  and  the  sensibility  schemes  of  Edinger,  Head,  and  Seiffer. 

TABLE  FOR  THE  GENERAL  DETERMINATION  OF  THE  PLACE  OF  TRANSVERSE 

LESIONS  {After  Schultze) 

Motor  Sensory 


Transverse  injuries  of  the 
cauda  equina,  cause: 


Flaccid  paralyses  in  the 
sciatic  region  and  the 
sacral  nerves  only,  or  also 
in  the  crural  or  obturator 


Awzsthesias  in  the  same 
areas;  in  individual  parts, 
also  parcesthesias,  hyper- 
cesthesias  and  pains. 


Transverse  injuries  of  the 
lumbar  enlargement,  cause: 


According  to  the  place  of 
the  injury  flaccid  paraly- 
sis in  the  sacral  nerves 
only,  or  also  in  the  lum- 
bar (crural,  obturat). 


Like  the  above. 


Transverse  injuries  of  the 
dorsal  segment,  cause: 


Paralysis  of  the  lower 
extremities  and  individ- 
ual parts  of  the  trunk, 
in  lesions  of  the  upper- 
most parts  also  oculo- 
pupillary  symptoms. 
Mostly  spastic  paralysis. 


Anesthesias  of  the  low- 
er extremities  and  sym- 
metrical parts  of  the 
trunk.  At  the  upper 
boundary  also  hyperes- 
thesias and  pains. 


Transverse  injuries  of  the 
cervical  enlargement,  cause: 


Spastic  paralysis  of  the 
lower  extremities;  paraly- 
sis of  the  muscles  of  the 
trunk;  partial  or  com- 
plete flaccid  or  atrophic 
paralysis  of  the  muscles 
of  the  upper  extremity. 


Anaesthesias  of  the  low- 
er extremities  of  the  trunk 
up  to  the  height  of  the 
shoulder,  partly  or  entire- 
ly of  the  upper  extremities. 
Hyperaesthesias  and 
pains. 


Transverse  injury  of  the 
uppermost  cervical  segment, 
causes: 


Usually  immediate  death,  or  if  the  injury  is  at  the 
lower  part  and  with  paralysis  of  the  phrenicus,  fatal 
termination  after  days  or  weeks. 

Radiating  pains  espe- 
cially in  the  n.  occipit. 
major. 


In  respect  to  bladder  and 
bowels  and  sexual  function. 


In  respect  to  reflexes. 


In  respect  to  trophic  dis- 
turbances. 


Severe   paralysis, 
cystitis.     Impotence. 


Later  Loss  of  the  cutaneous 

and  tendon  reflexes  in  the 
nerve  areas  concerned. 


Reaction  of  degenera- 
tion and  wasting  of  the 
muscles.  Decubitus:  tro- 
phic disturbances  of  the 
skin,  joints  and  bones. 


372 


DISEASES  OF  THE  SPINAL  CORD 


Bladder,  bowels,  and  sex- 
ual function. 


Reflexes. 


Trophic  disturbances. 


The  same  as  above.    Im- 
potence. 


The  same  as  above. 


The  same  as  above. 


The  same  as  above.    Im- 
potence. 


Retention  or  exagger- 
ation of  the  tendon  and 
cutaneous  reflexes.  Some- 
times, also,  absence  of  the 
tendon  reflexes. 


No  reaction  of  degen- 
eration. Often  marked 
diminution  of  the  electric 
excitability.    Decubitus. 


The  same  as  above.  Im- 
potence; sometimes  pria- 
pism. 


The  same  as  above. 


Reaction  of  degenera- 
tion in  the  muscles  of  the 
upper  extremities  other- 
wise as  above.  Often  rise 
in  temperature. 


Diagnosis. — After  the  first  effect  of  the  shock  is  over,  differentiation 
between  a  total  and  a  partial  transverse  lesion  is,  as  a  rule,  possible  without 
much  difficulty.  In  the  total  cases,  permanent,  complete,  motor  and  sensory 
paralysis  is  present  without  any  symptom  of  irritation  in  the  paralyzed 
region,  while  in  the  partial  cases,  incomplete  paralysis,  only  partial  sensory 
disturbance,  motor  and  sensory  irritation  phenomena  and  increased  vesical 
and  rectal  tenesmus  are  found. 

On  the  other  hand  to  determine  whether  a  break  in  conduction  depends 
upon  a  complete  or  a  partial  destruction  of  the  nerve  substance,  or  whether 
it  is  merely  a  matter  of  the  effects  of  a  simple  compression,  is  more  difficult 
and  under  some  circumstances  impossible.  It  is  certain  that  a  total  inter- 
ruption of  conduction  may  even  be  produced  by  a  lesion  causing  pressure. 
Likewise,  concussion  of  the  spinal  cord,  through  hemorrhages  into  its 
substance,  may  cause,  even  without  greater  anatomic  changes  in  it/  severe 
functional  disturbances,  which  may  simulate  a  contusion.  In  either  case 
rapid  improvement  frequently  occurs.  As  a  rule,  definite  differentiation 
between  a  crushing  of  the  cord  and  simple  compression  is  made  possible 
only  by  long  observation.  The  longer  duration  of  the  symptoms  of  func- 
tional loss  without  signs  of  improvement  makes  probable  the  presence  of 
irreparable  lesions  of  the  cord. 

Prognosis. — This  is  serious  in  all  injuries  of  the  spinal  column,  in  which 
the  spinal  cord  is  affected.  Total  transverse  injuries  are  fatal  without 
exception,  if  not  in  direct  connection  with  the  traumatism,  then  later  as  a 
result  of  the  unavoidable  complications  of  decubitus  and  cystitis.  Nor 
is  the  prognosis  in  partial  lesions  of  the  cord  quoad  vitam  always  favorable, 
since  a  long  time  may  elapse  before  there  is  functional  restoration  and 
especially  before  adjustment  of  the  disturbances  with  respect  to  the  bladder 
and  bowels,  while  the  patient  may  meanwhile  die  in  consequence  of  a  decu- 


FOCAL  DISEASES  OF  THE  SPINAL  CORD  373 

bitus  or  of  a  cystitis.  The  prognosis  is  made  still  worse  by  the  fact,  that 
hand  in  hand  with  the  improvement  of  the  at  first  severe  phenomena  of 
paralysis,  obstinate  irritation  symptoms  are  caused  by  the  sclerotic  processes 
following  closely  upon  the  destruction  of  tissue,  which  may  disturbrcon- 
siderably  the  ability  to  use  the  limbs,  which  otherwise  has  been  fortunately 
regained. 

Treatment. — This  in  the  days  immediately  following  an  injury  will 
have  to  be  restricted  to  preventing  further  displacement  of  the  broken  or 
dislocated  vertebras,  by  careful  placing  in  a  recumbent  position  and  the 
avoidance  of  all  movements.  Beside  this  all  measures  to  prevent  a  decu- 
bitus are  of  the  greatest  importance.  Regarding  operative  results  and 
especially  as  to  the  proper  time  for  operative  interference,  views  differ  con- 
siderably. The  majority  reject  an  early  operation  (before  the  sixth  week), 
since  in  the  incipient  period  the  phenomena  caused  by  the  commotion  and 
intra-medullary  hemorrhages  can  not  be  distinguished  from  those  due  to 
crushing,  contusion  or  compression. 

If  the  symptoms  of  a  total  transverse  lesion  persist,  operative  interference 
is  useless.  In  cases  of  incomplete  interruption  the  spontaneous  improve- 
ments are  often  very  extensive.  But  if  no  progress  is  clearly  discernible 
after  some  weeks,  this  indicates  rather  that  the  spinal  cord  is  injured  not  only 
by  a  transitory  lesion  due  to  pressure  but  that  a  permanent  compression  from 
detached  bone  splinters,  or  displaced  vertebrae,  is  present. 

Since  in  such  cases  there  is  a  possibility  of  effecting  an  improvement 
or  a  cure  by  removing  the  pressure,  an  operation  is  advisable. 

II.  The  Pressure  Paralyses  of  the  Spinal  Cord 

As  an  important  cause  of  either  quickly  or  slowly  appearing  compression 
paralyses  of  the  medulla  spinalis,  are  to  be  considered  not  only  the  tumors 
of  the  spinal  cord  and  its  membranes,  and  the  chronic  thickenings  of  the 
spinal  coverings,  which  similarly  to  pachymeningitis  cervicalis  hypertrophica, 
act  locally  like  a  tumor,  but  also  and  more  especially  the  diseases  of  the 
individual  vertebra. 

We  observe  suddenly  developing  pressure  paralyses  after  vertebral 
fractures  and  dislocations.  Among  the  changes  in  the  spinal  column  that 
effect  a  slow  gradual  compression  of  the  spinal  cord,  vertebral  caries  plays 
the  main  role.  In  the  great  majority  of  cases  it  is  tuberculous;  but  other 
forms  are  known — spondylitis  following  traumatism,  typhoid,  pneumonia, 
and  an  acute  osteomyelitis  of  the  vertebrae. 

More  rarely  than  by  tuberculous  caries,  the  spinal  cord  is  injured  by 
neoplasms  of  the  vertebrae  of  a  primary  or  a  metastatic  nature  or  by  malignant 
tumors,  which  starting  from  the  neighborhood  of  the  spinal  column,  extend 
to  it  and  the  spinal  cord.  Syphilis  also  can  cause  compression  of  the  cord 
by  exostoses  upon  the  body  of  the  vertebra  and  its  processes. 


374  DISEASES  OF  THE  SPINAL  CORD 

(a)  Caries  of  the  Spinal  Column,  Spondylitis  Tuberculosa 

Tubercular  vertebral  disease  occurs  at  all  periods  of  life,  but  especially  in 
childhood  and  adolescence.  It  may  appear  as  the  only  clinical  symptom  of 
tuberculosis;  but  usually,  other  signs  of  an  arrested  tuberculosis,  or  of  one 
still  active  are  demonstrable.  Traumatisms  of  the  spinal  column  may  play 
a  role  in  the  origin  of  caries,  in  that  localization  of  tubercular  processes  in 
the  vertebrae  is  favored  by  a  fall,  blow  or  contusion  in  tuberculous  individuals. 

Pathological  Findings. — The  seat  of  the  tubercular  granular  tissue, 
which  slowly  consumes  the  substance  of  the  bone,  is  particularly  in  the 
body  of  the  vertebra;  less  frequently  the  process  begins  in  the  vertebral 
joints,  intervertebral  discs  or  arches.  If  the  vertebral  bone,  destroyed  by 
caries,  is  gradually  compressed  by  the  weight  of  the  body,  the  consequence 
is  a  separation  of  the  spinous  processes,  a  sharp-angled  projection  or  lateral 
divergence  of  the  spinous  process  of  the  diseased  vertebra,  a  so-called  Pott's 
kyphosis.  This  may  appear  quite  suddenly,  if  through  a  direct  traumatism, 
or  through  severe  physical  exertion,  as  in  lifting  loads,  etc.,  the  diseased 
vertebra  suddenly  breaks. 

The  tuberculous  process  does  not,  as  a  rule,  remain  limited  to  the  bone; 
after  a  time  there  is  an  accumulation  of  cheesy  purulent  substance  between 
it  and  the  dura  and  the  latter  itself  undergoes  inflammatory  changes,  a  peri- 
pachymeningitis develops;  from  the  pressure  of  the  dura  mater,  as  a  result  of 
the  cheesy  substance  and  its  inflammatory  thickening,  the  spinal  cord  and  its 
roots  are,  as  a  rule,  considerably  compressed.  A  direct  transference  of  the 
tubercular  inflammation  to  the  spinal  cord  itself  through  the  dura  or  by 
the  way  of  the  root  bundles  and  blood-vessels,  rarely  takes  place. 

As  a  rule,  a  distinct  constriction  and  diminutio-n  in  size  of  the  spinal  cord 
is  to  be  seen  at  the  point  of  compression.  A  microscopic  examination  may 
disclose  comparatively  slight  changes  in  tissue,  even  though  during  life  serious 
disturbances  in  conductivity  existed.  In  such  cases  groups  of  swollen  axis 
cylinders  and  medullated  sheaths  with  incipient  phenomena  of  degeneration 
may  be  seen  in  the  midst  of  well-preserved  nerve  fibres;  the  glia  tissue  also 
shows  infiltration  phenomena  and  seems  thickened.  The  absence  of  inflam- 
matory changes  proper  is  emphasized  by  the  majority  of  authors.  If  the 
process  has  gone  farther,  wide-meshed  spaces  have  been  formed  through  the 
degeneration  of  nerve  fibres,  which  are  filled  with  granular  products  of 
degeneration  and  granular  cells.  In  the  later  stages  after  the  pressure  has 
been  exerted  quite  a  long  time,  it  comes  through  glia  proliferation  to  the 
formation  of  sclerotic  focal  lesions  in  place  of  the  destroyed  nervous  tissue. 
Secondary  ascending  and  descending  degenerations  may  follow  in  their  wake. 

Phenomena  of  the  Disease 
To  the  early  symptoms  of  a  developing  caries,  there  belongs  not  infre- 
quently a  dull  back-ache  caused  by  the  vertebral  disease ;  it  is  usually  localized 


CARIES  OF  THE  SPINAL  COLUMN  375 

to  a  definite  place  and  is  often  felt  only  after  motion,  through  concussions, 
and  by  direct  pressure.  A  symptom  that  results  from  the  pains  that  appear 
upon  motion,  is  a  certain  stiffness  of  the  spinal  column,  which  is  especially 
apparent  upon  bending,  as  the  patients  will  sink  upon  their  knees  to  spare 
the  spinal  column.  But  this  local  painfulness  of  certain  vertebrae  increasing 
upon  pressure,  is  by  no  means  a  regular  phenomenon;  it  is  occasionally 
absent  even  when  a  sharp-angled  kyphosis,  or  a  lateral  divergence  of  a  spinous 
process  can  already  be  demonstrated;  they  are  the  most  important  symptoms 
of  a  caries.  This  change  in  form  of  the  spinal  column  is  most  prominent  in 
the  dorsal  portion,  whereas  in  a  caries  of  the  cervical  and  lumbar  portions, 
it  may  be  recognized  at  times  only  as  a  flattening  of  the  normal  curvature. 
Since  the  vertebral  deformity  as  well  as  the  pains  may  be  absent  during  the 
entire  course  of  the  disease,  the  possible  proof  of  the  burrowing  of  pus  from 
the  diseased  vertebrae  is  of  great  diagnostic  importance.  These  so-called 
burrowing  abscesses  in  caries  of  the  upper  cervical  vertebrae  appear  occasion- 
ally in  the  space  between  the  posterior  pharyngeal  wall  and  the  spinal 
column — in  caries  of  the  lower  cervical  and  dorsal  vertebrae,  following  the 
course  of  the  psoas  muscle  in  the  inguinal  region;  a  rupture  into  the  thoracic 
cavity  has  also  occasionally  been  observed. 

Root  and  Spinal  Cord  Symptoms 

These  are  not  infrequently  wholly  absent,  even  in  the  presence  of  clin- 
ically pronounced  symptoms  of  caries — pains,  stiffness,  gibbosity — which  is 
not  at  all  remarkable,  since  it  is  not  the  bending  of  the  spinal  column  that 
causes  the  appearance  of  compression  phenomena,  but  only  the  narrowing 
of  the  spinal  canal,  through  the  thickening  of  the  dura  and  the  accumulation 
of  cheesy  tuberculous  masses  between  the  dura  and  the  bones,  which  need 
not  be  present  in  every  case.  But  it  happens  also  that  even  though  the 
vertebral  phenomena  are  present  only  in  slight  degree,  already  pronounced 
spinal  disturbances  are  demonstrable. 

As  the  first  sign  of  compression  root-irritation  symptoms  appear,  which 
are  of  a  neuralgic  nature  and  according  to  the  seat  of  the  injury,  may  appear 
as  uni-  or  bilateral  occipital,  branchial,  intercostal,  crural  or  sciatic  neuralgia. 
Not  infrequently  there  exist,  at  the  same  time,  paresthesias  of  great  variety. 
Sensory  phenomena  of  functional  loss,  hypcesthesias  and  anesthesias  appear 
only  later,  when  the  nerve  roots  are  destroyed  to  a  greater  extent.  Atrophic 
paralyses  of  definite  muscular  regions,  through  the  destruction  of  the  apper- 
taining anterior  roots,  also  develop,  only  later  as  a  rule. 

If  in  the  progress  of  the  disease,  the  cord  itself  is  injured  by  the  increasing 
pressure,  this  is  shown  as  a  rule  by  the  increased  exaggeration  of  the  tendon 
reflexes  lying  below  the  place  of  the  compression.  Even  before  the  appear- 
ance of  paralytic  phenomena,  patellar  and  ankle  clonus,  and  the  Babinski  sign 


376  DISEASES  OF  THE  SPINAL  CORD 

are  present.  The  motor  paralysis  of  the  legs,  which  next  appears  either 
quickly  or  slowly  in  a  caries  situated  above  the  lumbar  segment,  is  at  first 
flaccid  and  later  spastic. 

Disturbances  in  sensation  may  be  absent  for  a  strikingly  long  time  in  caries, 
or  be  but  slight,  even  though  a  pronounced  motor  paraplegia  is  already 
present.  An  attempt  has  been  made  to  explain  this  by  the  fact  that  the 
indurated  peripachymeningitis  compresses  especially  the  pyramidal  tracts 
from  the  front  and  the  sides,  while  the  posterior  columns  are  spared  more,  and 
that  just  as  in  lesions  of  the  peripheral  nerves,  resulting  from  pressure,  the 
sensory  fibres  have  greater  ability  to  resist  the  pressure  than  the  motor 
fibres.  But  in  a  more  severe  injury  of  the  cord  from  pressure,  a  decided 
sensory  disturbance,  affecting  all  qualities,  will  gradually  appear  in  caries 
also — a  disturbance  that  occasionally  is  bounded  by  a  rather  sharp  upper 
limit,  and  extends  to  the  region  of  the  roots,  which  originate  from  the 
diseased  segments  of  the  spinal,  cord.  In  rare  cases,  the  Brown-Sequard 
symptom  complex  has  also  been  observed  in  caries  (Oppenheim). 

Disturbances  of  the  bladder  and  bowels  are  occasionally  seen  early  along 
with  exaggeration  of  the  tendon  reflexes.  They  are  never  absent,  when 
the  motor  and  sensory  phenomena  of  paralysis  indicate  a  more  extensive 
transverse  lesion.     In  such  cases,  bed-sores  also  may  easily  develop. 

The  general  symptomatic  picture  of  caries  (which  has  just  been  discussed) 
will  exhibit  certain  characteristic  peculiarities,  according  to  the  seat  of  the 
carious  vertebral  change  in  the  upper  and  lower  cervical  portion  of  the 
vertebral  column  and  in  the  lower  dorsal  and  upper  lumbar  vertebrae.  These 
peculiarities  we  shall  have  to  discuss  briefly. 

i.  In  caries  of  the  upper  cervical  vertebrae  and  of  the  atlanto-occipital 
joint,  beside  pains  and  stiffness  in  the  back  of  the  neck,  a  neuralgia  of  the  occip- 
ital nerves  on  one  or  both  sides  is  the  most  important  early  symptom.  In 
all  changes  of  position  of  the  upper  part  of  the  body,  the  patient  supports 
his  head  with  his  hands  (Rust's  symptom).  Oppenheim  mentions  also  the 
occurrence  of  paralysis  of  the  accessory  nerve  and  unilateral  atrophy  of  the 
tongue;  upon  compression  of  the  medulla  oblongata  bulbar  symptoms  are 
added;  where  pressure  lesions  are  produced  in  the  upper  cervical  segments, 
phrenic  phenomena  will  be  noted  in  some  cases.  There  are,  besides,  spastic 
paresis  of  the  arms  and  legs  and  paralyses  of  the  musculature  of  the  trunk, 
as  well  as  sensory  disturbances  extending  to  the  neck. 

2.  If  the  cervical  enlargement  is  affected  in  disease  of  the  lower  cervical 
vertebrae,  this  may  be  recognized  by  the  fact  that  beside  spastic  paresis  of 
the  legs  and  weakness  of  the  musculature  of  the  trunk,  a  flaccid  paralysis  of 
the  muscles  of  the  arm  and  hand  develops  with  muscular  atrophy  and  electrical 
reaction  of  degeneration. 

3.  A  caries  of  the  eleventh  and  twelfth  dorsal  vertebrae  and  of  the  first 
lumbar  vertebra  results  in  a  pressure  lesion  of  the  lumbar  enlargement. 


CARIES  OF  THE  SPINAL  COLUMN  377 

The  paralysis  of  the  legs  is,  therefore,  from  the  first,  flaccid  and  atrophic 
with  signs  of  electric  reaction  of  degeneration;  at  the  same  time,  the  tendon 
reflexes  are  markedly  diminished  or  even  lost.  The  sensory  disturbance  re- 
mains restricted  to  the  legs;  bladder  and  bowels  usually  show  considerable 
disturbances. 

Diagnosis. — The  diagnosis  of  a  compression  paralysis  is  easily  made, 
if  besides  the  root  and  cord  symptoms,  which  indicate  a  progressive  transverse 
lesion,  a  deformity  of  the  spinal  column  with  local  sensitiveness  to  pressure, 
may  be  demonstrated.  Then  only  the  further  differential  diagnostic 
determination  is  necessary  as  to  whether  a  tubercular  process  is  the  basis 
of  the  vertebral  affection,  or  if  a  tumor  of  the  spinal  column,  especially  a 
carcinoma  is  to  be  assumed.  The  exclusion  of  the  usually  rare  vertebral 
disease  following  traumatism,  typhoid,  acute  osteomyelitis,  will,  as  a  rule, 
be  easy  by  taking  into  consideration  the  aetiological  factors. 

The  matter  may  be  far  more  difficult  if,  as  not  rarely  occurs,  symptoms 
that  point  to  a  disease  of  the  spinal  column  are  absent  for  a  longer  time  and 
only  the  signs  of  an  increasing  transverse  disease  of  the  cord  are  present. 
Under  such  circumstances  positive  differentiation  between  tumors  of  the 
spinal  cord,  the  spinal  membranes  and  myelitis  having  a  syphilitic  or  a 
tubercular  basis  on  the  one  hand,  and  a  caries  or  a  vertebral  tumor  on  the 
other,  is  absolutely  impossible  at  first  and  becomes  possible  only  after  long 
observation. 

For  the  diagnosis  in  favor  of  caries,  in  the  first  place,  the  proof  of  other 
signs  of  recent  or  arrested  tuberculosis  {fever,  enlarged  glands,  affection  of  bones) , 
must  be  considered.  Occasionally  also  it  is  possible  to  establish  destruction 
or  displacement  of  the  vertebrae  by  means  of  the  X-rays.  Furthermore  the 
ascertainment  of  burrowing  abscesses  may  be  decisive;  value  has  also  been 
laid  upon  the  greater  stiffness  of  the  spinal  column  in  caries  in  contradistinc- 
tion to  tumors  of  the  spinal  cord.  Even  if  it  is  certain,  that  larger  tumors  of 
the  spinal  membranes  may  run  their  course -without  special  hindrance  to 
the  motility  of  the  spinal  column,  this  holds  good  actually  only  for  tumors 
of  the  dorsal  part,  while  those  in  the  region  of  the  cervical  segment  occasion- 
ally cause  a  considerable  stiffness  in  the  neck. 

The  differential  diagnosis  in  regard  to  a  meningomyelitis  syphilitica 
must  be  especially  based  on  the  fact  that  in  this  disease,  the  symptoms  show 
quite  a  striking  variety  and  that,  as  a  rule,  the  picture  of  this  disease  points 
to  multiple,  spinal  and  also  cerebral  foci.  In  a  doubtful  case  the  success  of  a 
course  of  specific  treatment  may  shed  light  on  the  nature  of  the  disease. 

For  the  distinction  between  a  spondylitis  tuberculosa  and  vertebral  tumors, 
one  must  consider  that  the  latter  usually  attack  and  infiltrate  several  adjacent 
vertebrae  and  therefore  not  infrequently  cause  a  simple  sinking  into  each 
other  of  the  vertebrae  (changes  easily  seen  on  the  Roentgen  plates),  and  a 
diminution  in  height  of  the  patient;  also,  as  a  rule,  the  developing  gibbosity  is 


378  DISEASES  OF  THE  SPINAL  CORD 

less  angular  than  in  caries,  since  several  vertebrae  participate  in  the  deform- 
ity. The  age  may  afford  a  further  important  point,  inasmuch  as  in  the 
young,  the  assumption  of  a  vertebral  carcinoma  has  in  itself  little  probability, 
while,  on  the  other  hand,  in  more  advanced  age  with  a  more  complete  loss  of 
strength,  a  suspicion  of  vertebral  cancer  seems  justified  and  makes  it  advis- 
able to  seek  for  a  primary  cancerous  focus. 

Prognosis  and  Course. — The  tuberculous  bone  process,  which  may 
extend  over  years  without  spinal  phenomena  necessarily  occurring,  may  at 
any  time  come  to  a  standstill  and  recovery.  The  same  holds  true  of  the 
spinal  paralytic  symptoms,  which  even  after  persistence  for  months  and  years 
may  completely  disappear  again.  The  course  of  the  disease,  however,  is  but 
rarely  so  favorable.  In  the  majority  of  cases  of  caries,  with  compression  of 
the  spinal  cord,  death  results,  whether  through  progress  of  the  tuberculosis 
in  other  organs  or  by  the  unavoidable  results  of  a  decubitus  or  cystitis  in 
advanced  paralysis.  Sometimes  the  phenomena  of  the  spinal  cord  disappear 
only  in  part,  as  after  healing  of  the  bone  disease  and  the  inflammatory  processes 
in  the  membranes  the  sclerotic  changes  in  the  spinal  cord  have  already  ad- 
vanced so  far,  that  a  compensation  of  the  functional  disturbances  is  no  longer 
possible.  In  such  cases  as  permanent  signs  of  the  former  compression,  there 
are  more  or  less  pronounced  spastic  pareses  in  the  extremities,  possibly  com- 
bined with  disturbances  in  sensibility  and  of  bladder  function.  A  difficulty  in 
micturition  was,  in  one  of  my  cases,  after  the  disappearance  of  all  the  other 
spinal  symptoms,  for  years,  the  only  remaining  really  disturbing  phenomenon. 
The  prognosis  is  the  more  favorable,  the  less  the  phenomena  of 
compression  have  advanced  at  the  beginning  of  the  treatment  and  the 
younger  and  stronger  the  patient.  It  is  remarkable  that  relapses,  especially 
after  traumatism,  may  appear  at  any  time  and  that  even  after  persistence  of  a 
simple  gibbosity  for  decades,  spinal  symptoms  may  still  appear  occasionally 
afterwards. 

Treatment. — The  most  important  thing  in  every  caries  with  marked 
phenomena  of  compression  is  a  position  assuring  absolute  rest  for  the  diseased 
spinal  column.  Even  by  simply  lying  on  his  back  in  bed  for  a  long  period  of 
time,  the  patient  may  occasionally  bring  about  a  complete  cure  from  severe 
compression  paralysis.  In  addition  the  most  careful  general  nutrition  and 
care  of  the  skin  is  essential  for  the  prevention  of  bed-sores  (smooth  mattress, 
washing  the  places  where  pressure  occurs  several  times  daily  (alcohol 
and  water),  air  or  water  cushions). 

Very  rapid  improvement  of  the  paralytic  symptoms  is  observed  after 
careful  extension  of  the  spinal  column.  This  is  most  easily  performed  in 
caries  cervicalis,  as  the  pull  upon  the  head  is  effectually  exerted  by  Glisson's 
suspensory  apparatus  with  light  loads  (3  to  4  lbs.  at  the  beginning,  and  never 
exceeding  12  to  15  lbs.),  the  opposite  pull  being  effected  by  the  weight  of  the 
body,  the  bed  being  elevated  at  the  head.     Occasionally,  after  using  exten- 


CARIES  OF  THE  SPINAL  COLUMN  379 

sion,  a  deterioration  is  observed,  so  that  a  return  to  simple  rest  upon  the  back 
without  further  stretching  becomes  necessary. 

If  the  pressure  phenomena  are  but  slight  from  the  first,  or  if  the  paralysis 
has  disappeared  by  rest  in  bed,  and  the  general  health  and  strength  of  the 
patient  is  excellent,  ambulatory  treatment  with  a  well-fitted  plaster -of -Par  is 
corset  and  the  usual  supporting  apparatus  by  which  the  diseased  vertebra  is 
relieved  from  the  weight  of  the  parts  of  the  body  lying  above  it  may  be 
resorted  to  (Sayres  Jury  mast,  the  head  supports  of  Nebel,  Heusner,  Schede). 
Ambulatory  treatment  has  the  great  advantage  that  through  it  the  patient 
may  be  enabled  to  live  in  the  open,  in  the  fresh  air  (seaside  resort)  and  thus 
through  the  movement  and  exercise  of  his  musculature,  a  better  circulation, 
an  increase  in  appetite,  and  a  better  general  condition  are  attained. 

Operative  interference  has  been  suggested  and  attempted  in  such  cases, 
in  which  the  compression  paralysis  remains  the  same,  or  even  increases  under 
the  usual  extension  treatment.  It  is  followed  in  almost  every  instance  by 
an  immediate  favorable  result  since  the  phenomena  of  paralysis  improve 
immediately  after  the  tuberculous  focus  is  removed.  As  a  rule,  however, 
the  improvement  does  not  persist,  and  the  resection  of  the  vertebral  arches — 
in  the  case  the  tuberculosis  is  not  restricted  to  these  alone — has  many  more 
failures  than  successes  to  its  account.  At  any  rate,  Schede  considers  the 
operative  opening  of  the  spinal  canal,  as  a  last  resource,  not  only  per- 
missible, but  under  circumstances  even  necessary. 

The  course  prescribed  by  Calot  for  recent  and  old  cases,  forcibly  to  press 
back  under  anaesthesia  the  projecting  vertebra  and  to  fix  the  position  so 
attained  by  bandages,  has  been  abandoned  as  being  too  dangerous. 

Of  local  measures,  rubbing  with  soft  soap,  and  painting  with  iodine 
have  been  frequently  used.  Oppenheim  saw  a  favorable  influence,  several 
times,  from  iodide-iron  preparations. 

For  combating  spastic  conditions,  but  also  in  paralysis  without  severe 
muscular  spasms,  if  the  patients  can  be  moved,  the  use  of  salt  and  brine 
baths  and  an  eventual  "bath  cure"  in  Nauheim  and  Kreuznach  are  to  be 
recommended. 

Electric  treatment,  at  best,  should  be  confined  entirely  to  old  cases  that 
have  run  their  course,  with  flaccid  paralysis  and  atrophic  muscles.  Direct 
galvanic  treatment  applied  to  the  back  is  productive  at  times,  according  to 
Oppenheim,  even  in  old  cases,  of  favorable  effects. 

(b)  Carcinoma  and  Other  Tumors  of  the  Vertebral  Column 

The  not  infrequent  vertebral  cancer  is  nearly  always  metastatic,  and  is 
found  relatively  frequently  in  carcinoma  of  the  breast,  but  the  abdominal 
organs  must  also  be  taken  into  consideration  as  the  place  of  origin.  The 
connection  between  the  vertebral  spinal  cord  phenomena  and  cancer  remains 
quite  often  unsuspected  for  some  time  because  the  primary  cancerous  focus 


38o 


DISEASES  OF  THE  SPINAL  CORD 


at  first  causes  few  pronounced  clinical  symptoms,  or,  as  in  carcinoma  of  the 
breast,  under  certain  conditions,  it  has  been  removed  by  operation  some  time 
before  the  appearance  of  the  disease  of  the  spinal  cord.  As  a  consequence, 
doubts  as  to  its  nature  may  exist  for  some  time.     Vertebral  cancer,  like  most 

of  the  other  tumors,  which,  beginning 
either  in  the  vertebrse  themselves  or 
in  their  neighborhood,  attack  the 
vertebras,  effects,  as  a  rule,  several 
vertebral  bodies.  Owing  to  the  fact 
that  the  tumors  extend  to  the  verte- 
bral processes,  and  also  infiltrate  the 
soft  parts  enveloping  the  spinal 
column,  there  arise  occasionally  large, 
easily  palpable  excrescences  on  the 
spinal  column.  As  a  rule,  however, 
this  is  not  the  case,  and  as  a  direct 
consequence  of  vertebral  destruction, 
g  we  find  only  a  sinking  of  one  body  into 
the  other  (Fig.  82).  Besides  carci- 
noma, which  is  by  far  the  most  fre- 
quent and  therefore  the  most  impor- 
tant tumor  of  the  spinal  column,  there 
have  been  described  sarcomata,  osteo- 
sarcomata,  the  rare  enchondromata, 
the  multiple  appearing  myelomata, 
also  gummata  and  echinococcus  cysts. 

Syphilis  also  leads  to  the  forma- 
tion of  exostosis  on  the  body  of  the 
vertebra  and  its  processes. 

Symptomatology. — The   disease 

Fig.  82—  Carcinoma  of  the  vertebral  column  picture  shows  great  similarity  to  that 

st"  S-SSS'vSSta? jtS^K  of  f  ^"ndylitis  tuberculosa.    In  the 

tumor  and  a  narrowing  of  the  vertebral  canal  typical  cases,  there  are  found  besides 
due  to  this.     (After  Schlesinger.)  .     .  ,  ,,  ,        . 

obvious  changes  in  the  spinal  column 
with  local  vertebral  pain,  which  is  increased  upon  motion,  root  irritation 
symptoms  and  phenomena  of  compression  of  the  cord.  Demonstrable  changes 
in  the  bones,  however,  may  be  absent  in  carcinoma  at  a  time  when  root  and 
cord  symptoms  are  already  pronounced.  As  an  important  bone  symptom, 
there  appears  occasionally  a  gradual  lessening  of  the  length  of  the  body 
through  the  sinking  together  of  several  vertebras  or  a  gibbosity,  which  be- 
cause of  the  disease  in  several  vertebras,  is  usually  less  pointed  than  in 
caries.  More  rarely  it  comes  to  the  development  of  large  palpable  thicken- 
ngs  of  the  vertebras. 


£r 


'■"■  ■ 


TUMORS  OF  THE  VERTEBRAL  COLUMN  381 

The  pains,  not  only  the  local  pains,  but  the  neuralgic  pains  produced  by 
root  compression  as  well;  are  in  vertebral  tumors,  as  a  rule,  extraordinarily 
violent  in  contrast  to  those  due  to  caries.  The  spontaneous  pain  is  not 
rarely  very  severe,  though  the  local  tenderness  to  pressure,  which  even  in 
extended  vertebral  carcinoma  may  be  restricted  to  several  vertebral  spinous 
processes,  is  but  slight.  The  cord  symptoms,  motor  and  sensory  paralyses, 
and  disturbances  in  bladder  and  rectum,  develop  rather  quickly  upon  occasion, 
when  a  carcinoma  after  breaking  through  the  dura,  invades  the  cord.  With 
advanced  changes  in  the  bones,  a  slight  traumatism  may  suffice  to  give  rise 
to  a  sudden  collapse  of  the  vertebras  and  an  acute  paraplegia. 

Since  in  the  paraplegic  limbs,  the  pains  usually  persist  with  great  violence 
Cruveilhier  long  ago  spoke  of  a  paraplegia  dolorosa  specially  in  vertebral 
carcinoma. 

The  course  of  the  disease  is  rapid  in  the  majority  of  cases;  especially  in 
sarcomata  and  carcinomata  the  disease  rarely  lasts  longer  than  nine  months 
to  one  and  one-half  years. 

Diagnosis. — Even  if  a  deformity  of  the  spinal  column  points  to  a  com- 
pression paralysis,  differentiation  from  a  caries  will  not  always  be  easy. 
The  diagnosis  of  vertebral  cancer  will  have  to  be  based  on  the  age  of  the 
patient,  which  justifies  a  suspicion  of  carcinoma,  upon  general  loss  of  strength, 
above  all,  on  the  demonstration,  of  a  primary  cancerous  focus  in  other  organs 
and  on  a  history  of  cancer,  extirpation  of  the  breast,  etc.  On  the  other 
hand,  youth  and  the  proof  of  recent  or  healed  tuberculosis  speak  for  a 
diagnosis  of  caries.  It  is  important  for  differential  diagnosis,  that  in  carcino- 
matoses also  spinal  phenomena  may  be  observed,  which  depend  upon  a 
disseminated  myelitis.  In  the  absence  of  definite  changes  in  the  bones, 
and  of  stronger  root  irritation  symptoms,  with  the  demonstration  of  a  primary 
tumerous  focus,  we  must  reckon  with  this  possibility.  An  X-ray  investiga- 
tion, which  should  always  be  tried  as  soon  as  possible,  will  be  of  further 
assistance  in  these  cases  also. 

Treatment. — If  there  be  a  suspicion  of  a  syphilitic  affection  of  the  bones, 
with  the  formation  of  an  exostosis  leading  to  compression,  specific  treatment 
is  to  be  begun,  which  in  a  series  of  cases  (Oppenheim,  Leyden,  and  others) 
led  to  complete  recovery.  Operative  interference  is  only  to  be  advised  when 
the  tumor  is  not  a  metastatic  growth.  In  the  primary  tumors  of  the  spinal 
column,  a  favorable  result  can  be  attained  by  means  of  an  operation,  at  least 
temporarily,  as  it  ameliorates  the  torturing  pains.  Recurrence  occurs 
quickly  as  a  rule  in  consequence  of  the  usually  great  extension  of  the 
tumors  over  several  vertebrae.  But  according  to  isolated  observations  in 
osteoma  and  sarcoma  (Oppenheim)  a  cure,  which  may  last  for  years,  seems  to 
lie  within  the  bounds  of  possibility,  so  that  one  must  not  deprive  the  patient, 
if  conditions  are  favorable,  of  his  chance  by  an  operation,  though  the  prog- 
nosis is  usually  hopeless.     In  the  majority  of  cases,  we  shall  have  to  limit 


382 


DISEASES  OF  THE  SPINAL  CORD 


ourselves  to  combating  the  pains  (morphine,  aspirine,  pyramidon) ,  and  by  a 
careful  recumbent  position  and  avoidance  of  all  unnecessary  movements  try  to 
attain  a  permanent  relief  for  the  diseased  bones. 

(c)  Neoplasms  of  the  Spinal  Cord  and  its  Membranes 
Pathological  Anatomy. — Tumors  of  extra-medullary  development  are 
more  common  than  neoplasms  arising  in  the  spinal 
cord  itself.  Among  the  extra-medullary  tumors 
those  situated  outside  of  the  dura  mater,  if  we  except 
those  of  the  vertebrae,  are  much  rarer  than  the  prac- 
tically much  more  important  intra-dural  tumors.  In 
the  rare  extra-dural  neoplasms,  it  is  usually  a  matter  of 
lipomata  and  echinococci;  among  the  tumors  develop- 
ing within  the  dura,  fibromata,  sarcomata  and  their 
mixed  forms  play  the  most  practically  important  part, 
since  they  usually  occur  in  circumscribed  form  about 
the  size  of  peas  to  hazel-nuts  and  are  easily  removed 
by  operation;  besides  these  are  found  intra-dural 
myxomata,  psammomata,  less  frequently  lipomata, 
adenosarcomata,  lymphangiomata,  and  teratomata. 
Sarcoma  occasionally  appears  in  multiple  form,  or  it 
spreads  diffusely  over  the  membranes  of  the  spinal 
cord,  the  individual  growths  varying  much  in  size. 

The  greater  number  of  the  growths  in  the  spinal 
cord  itself  are  gliomata;  they  are  preferably  situated 
in  the  cervical  and  upper  dorsal  cord  and  in  the  upper 
part  of  the  lumbar  enlargement;  other  neoplasms  oc- 
curring are  sarcomata,  tubercles  and  gummata.  Mul- 
tiple neurofibromata  emanating  from  the  roots  of  the 
spinal  cord  are  frequently  only  a  partial  symptom  of 
an  extensive  general  neurofibromatosis  of  the  nervous 
system. 

Causes. — Excluding  the  parasitic  and  infectious 
growths  we  know  nothing  of  the  aetiology  of  the  neo- 
plasms and  must  be  satisfied  with  the  assumption  of 
an  abnormal  congenital  predisposition  of  the  tissues. 
It  seems  that  traumatisms  are  to  be  considered  as  excit- 


FiG.    83.  —  Intra-dural 

tumor,    situated  opposite  ing  factors  in  the  development  of  the  growths  or  that 

the    dorsal    cord  between  ,-,  ,  r      .  f  .  .•■     .  .   1 

the  6th  and  7th  pair  of  tnev    are    at    least    factors    favoring    their    quicker 

dorsal  roots,  compressing 
the  spinal  cord  posteriorly 
from  the  right  side. 
Drawing  is  natural  size 
after  cutting  open  and 
spreading      the     dura. 

( Author 's  own  observation) .  emanating  from  the  membranes  are  not  malignant 


progress. 

Tumors  in  the  Membranes  of  the  Spinal  Cord 
Symptomatology. — Since  the  majority  of  tumors 


TUMORS  OF  THE  SPINAL  MEMBRANES 


383 


and  grow  but  very  slowly,  the  appearance  of  symptoms  of  compression 
in  the  substance  of  the  spinal  cord  is  preceded  by  an  earlier  stage  of 
neuralgic  pains,  which  may  extend  over  months  and  even  years  and 
which  are  caused  by  the  irritation  due  to  pressure  upon  the  posterior 
roots.  These  radiating  pains  are  localized  to  one  or  both  sides  of  a 
definite  nerve  region  and  pre- 
sent the  picture  of  an  inter- 
costal or  of  a  sciatic  neuralgia, 
or  a  neuralgia  of  the  brachial 
plexus.  Patients  frequently 
complain  of  an  increase  in 
the  pain  whenever  the  spinal 
column  is  jarred,  as  in  cough- 
ing, sneezing,  or  by  pressing 
upon  it.  The  prodromal 
stage  of  pains  need  not  always 
be  present  in  tumors  of  the 
spinal  cord  membranes;  oc- 
casionally the  pain  is  present 
to  only  a  slight  degree,  or  lasts 
only  a  short  time.  Irritation 
symptoms  in  the  anterior 
motor  roots,  tremors,  mus- 
cular contractions,  spasms  oc- 
cur but  rarely  compared  to  the 
sensory  irritation  phenom- 
ena, even  where  a  gradually 
growing  tumor,  as  shown  by 
its  location,  must  have  pressed 
almost  equally  on  posterior 
and  anterior  roots.  As  a  rule, 
in  a  compression  of  the  an- 
terior roots,  the  irritation  FlG-  84—  Gower's  scheme  (from  the  7th  cervical  to  the 
.  2nd  lumbar  segment)    shows  the  relation  of  the  spinous 

phenomena     are    quickly    fol-  processes  and  of  the  places  of  exit  of  the  roots  to  the 

lowed  by  atrophic  paralysis  in  £*■*  r^tSii"1  t£  *h  S^S 

the     corresponding     muscular  is  regarded  as  diseased. 

area. 

In  the  usual  location  of  a  tumor  in  the  dorsal  portion  of  the  cord,  atrophic 
muscular  paralyses  caused  by  pressure  lesion  of  the  motor  roots  fall  entirely 
into  the  background  of  the  clinical  picture,  since  weakness  and  wasting  of 
individual  intercostal  muscles  escape  detection.  Only  in  cases  in  which 
the  lower  dorsal  roots  (8-12  pair)  are  injured  by  the  tumor,  can  iini-  or 
bilateral  paralyses  of  the  abdominal  muscles  appear  and  with  the  sensory 


384  DISEASES  OF  THE  SPINAL  CORD 

phenomena  of  irritation,  form  an  early  local  symptom  of  considerable  diag- 
nostic importance. 

A  less  frequent  symptom  of  irritation,  which,  however,  must  be  sought 
for,  is  a  local  tender  point  of  the  spinal  column,  usually  at  the  height  of  those 
vertebrae,  opposite  to  which  the  tumor  is  located.  This  vertebral  tenderness 
is  best  sought  for  and  tested  in  this  manner:  exert  strong  pressure  with  the 
back  of  the  flexed  index  or  middle  finger  to  one  side  of  the  spinous  processes 
of  the  vertebrae.  This  will  be  found  painful  especially  on  the  side  which 
was  the  seat  of  the  neuralgia  at  the  onset. 

As  the  tumor  continues  to  grow,  the  signs  of  a  compression  of  the  cord 
are  added  to  the  symptoms  of  root  irritation,  which  persist  in  most  of  the 
cases;  a  motor  and  sensory  paralysis  which,  as  a  rule,  advances  slowly  and 
regularly  in  the  part  of  the  body  lying  below  the  place  of  pressure.  The 
motor  paralysis  is  always  spastic  when  the  tumor  is  located,  as  is  usually 
the  case,  above  the  lumbar  segment;  in  the  beginning  it  usually  appears 
as  slight  paresis  in  one  leg,  and  later  extends  to  the  other  side,  and  even 
when  paraparesis  is  already  present,  the  side  of  the  body  corresponding  to 
the  seat  of  the  tumor  usually  shows  the  more  severe  paralytic  phenomena. 

The  tendon  reflexes,  the  increase  of  which  occasionally  is  the  first  symptom 
of  compression  of  the  cord,  are  regularly  and  considerably  exaggerated; 
patellar  and  ankle  clonus  are  present  and  frequently  the  Babinski  sign, 
especially  in  the  more  markedly  paralyzed  limb.  The  reflexes  of  the  abdomi- 
nal muscles  are  usually  lost  or  weakened. 

The  disturbance  in  sensation  may  be  limited  at  the  beginning  to  one  leg, 
because  the  cord  at  first  is  compressed  on  one  side,  so  that  with  a  simul- 
taneous paresis  of  the  leg  corresponding  to  the  side  of  the  tumor,  there 
comes  to  exist  for  some  time  the  symptom  complex  of  the  Brown-Sequard 
unilateral  paralysis.  Thus,  for  instance,  Oppenheim  saw  a  thermancesthesia 
in  the  opposite  leg  precede  the  development  of  the  homolateral  paralysis 
in  two  of  his  cases.  As  the  transverse  lesion  increases  in  size,  due  to  the 
growing  tumor,  the  unilateral  sensory  paralysis  is  soon  transformed  into  a 
bilateral  disturbance;  it  may  not  come  under  observation  until  this  stage 
is  reached. 

In  the  typical  cases,  the  upper  boundary  of  the  sensory  disturbances, 
which  is  usually  a  horizontal  line  on  the  trunk,  may  be  established  quite 
accurately  upon  repeated  tests.  At  this  upper  margin,  sensibility  is  not 
completely  lost  but  only  diminished.  This  upper  hypasthetic  cutaneous 
area  gradually  passes,  as  we  go  downward,  over  into  the  region  of  the  skin 
that  is  more  or  less  decidedly  anasihetic.  In  some  cases,  it  has  even  been 
possible  to  demonstrate  an  abnormal  sensitiveness  in  the  skin  area  immedi- 
ately above  the  hypaesthetic  zone — a  hyperesthesia.  Especially  characteristic 
and  important  for  the  diagnosis  of  tumor  is  the  determination  that  the 
upper  boundary  of  disturbed  sensibility  remains  in  the  same  place  even 


INTRA-MEDULLARY  TUMORS  385 

after  a  longer  period  of  observation,  or,  at  any  rate,  undergoes  only  an 
unimportant  move  upwards.  This  depends  on  the  fact,  that  with  a  slowly 
growing  tumor,  even  a  slight  increase  in  its  thickness  must  cause  a  marked 
compression  in  the  narrow  spinal  canal,  whereas  a  slight  increase  in  length 
can  cause  no  perceptible  extension  of  the  sensory  disturbances  upwards 
by  lesions  of  the  roots  and  the  cord. 

Disturbances  of  bladder  and  bowels  are  always  present  as  soon  as  the 
phenomena  of  cord  compression  have  developed  to  a  somewhat  considerable 
degree;  but  even  in  the  beginning  of  the  disease  bladder  disturbances  in  the 
form  of  increased  urinary  pressure  or  of  slight  difficulty  in  micturition  may 
make  themselves  felt. 

If  the  tumor  is  not  located,  as  we  have  assumed  up  to  this  point  in  the 
discussion,  in  its  favorite  place  in  the  dorsal  portion  of  the  spinal  cord,  but  in 
the  region  of  the  cervical  or  lumbar  enlargement,  or  still  farther  down  in  the 
conus  terminalis  or  the  cauda  equina,  the  symptomatic  picture,  according  to 
the  location  in  one  of  these  regions  will  present  certain  peculiarities,  impor- 
tant for  localization.  In  compression  of  the  cervical  enlargement,  we  shall 
see,  in  addition  to  spastic  paresis  of  the  lower  extremities  after  inceptive 
neuralgic  troubles  in  the  course  of  the  nerves  of  the  arm,  flaccid  degenerative 
paralyses  of  the  arm-muscles  develop  caused  partly  by  the  destruction  of  the 
anterior  roots,  but  partly  also  by  injury,  through  pressure,  of  the  anterior  horn 
ganglion  cells  of  the  cervical  enlargement.  Through  irritation  or  paralysis 
of  the  sympathetic  fibres,  lying  in  the  lower  cervical  and  upper  dorsal  portion 
of  the  cord,  there  may  appear  also  so-called  oculopupillary  symptoms,  dilata- 
tion or  contraction  of  the  pupil  and  palpebral  fissure  on  one  or  both  sides. 
Tumors  of  the  lumbar  region,  because  of  the  fact  that  the  nerve  roots  are 
placed  close  to  one  another,  generally  cause  very  violent  and  extensive 
neuralgic  pains;  also  the  paralysis  that  appears  later  in  the  legs  is  from  the 
first  flaccid  and  atrophic  and  is  associated  with  loss  of  the  tendon  reflexes. 

The  symptomatic  pictures  evoked  by  compression  of  the  conus  and  the 
cauda  will  be  discussed  in  a  special  chapter. 

Intra-medullary  Tumors 

The  intra-medullary  tumors  in  their  symptomatology  and  course  fre- 
quently differ  essentially  from  the  typical  picture  of  the  tumors  of  the 
spinal  cord  membranes  discussed  above.  In  the  first  place,  a  pure  neuralgic 
stage  lasting  for  any  length  of  time  is,  as  a  rule,  absent  in  tumors  of  the  spinal 
substance.  Even  if  with  the  development  of  a  new  growth  in  the  periphery 
of  the  cord,  in  the  neighborhood  of  the  membranes  and  the  posterior  root 
zone,  pains  form  the  first  symptom,  nevertheless,  soon  afterwards,  or  even 
synchronously,  appear  the  signs  of  a  transverse  lesion,  during  which  a 
transitory  Brown-Sequard  type  of  paralysis  may  be  observed.  Likewise 
25 


386 


DISEASES  OF  THE  SPINAL  CORD 


the  pains  excited  occasionally  during  the  course  of  the  disease,  by  tumors 
of  the  cord,  whether  through  irritation  of  tracts  within  the  spinal  cord 
or  an  accompanying  chronic  meningitis,  are  usually  only  of  slight  degree, 
and  fall  quite  into  the  background  of  the  disease  picture. 

If  a  tumor  develops  from  the  first  in  the  central  portion  of  the  cord,  dis- 
ease pictures  may  develop,  which  do  not  at  all  resemble  those  of  tumor  of  the 
spinal  cord  membranes.  In  more  diffuse  extension  over  a  transverse  section 
of  the  spinal  cord,  there  appears  for  a  long  period  of  time,  the  picture  of 
a  subacute  or  chronic  myelitis  transversa,  without  any  demonstrable  cause; 
in  gliomata  with  simultaneous  cavity  formation,  the  phenomena  of  a  syringo- 
myelia present  themselves;  it  is  precisely  in  cord  tumors  that  dissociated 
sensory  paralysis  is  found  most  frequently;  destruction  of  the  anterior  horns, 

through  tubercles  or  a  sarcoma  with 
rapid  extension  in  the  longitudinal  axis 
produces  the  picture  of  a  rapidly  pro- 
gressing spinal  muscular  atrophy. 

For  the  course  of  tumors  of  the  cord, 
it  is,  moreover,  characteristic  to  a  cer- 
tain degree,  that  a  comparatively  rapid 
growth  in  the  longitudinal  direction  up- 
wards and  downwards,  takes  place, 
and  thereby  a  swifter  moving  of  the 
boundary  of  the  sensory  paralysis  up- 
wards is  observed  than  in  the  tumors  of  the  membranes. 

General  Diagnosis. — Differentiation  of  a  tumor  of  the  spinal  membranes 
from  other  diseases  of  the  spinal  cord  not  dependent  upon  compression, 
which  run  their  course  under  the  picture  of  a  diffuse  transverse  disease,  is 
not  as  a  rule  difficult.  Confusion  with  a  pure  myelitis  transversa  is  prevented 
by  the  usually  acute  onset  of  this  disease  without  preceding  neuralgic  dis- 
turbance, as  well  as  by  the  simultaneous  demonstration  of  causes  and 
assisting  factors  frequently  to  be  considered  in  acute  myelitis,  as  preceding 
infectious  diseases,  anginas,  colds,  over-exertions,  possibly  also  the  existence 
of  fever.  For  the  differential  diagnosis  from  multiple  sclerosis,  which  occa- 
sionally, for  some  time  presents  the  symptomatic  picture  of  a  chronic  trans- 
verse myelitis,  the  chief  weight  must  be  laid  upon :  i .  The  permanent  absence 
of  pains,  not  only  in  the  beginning  of  the  disease  but  throughout  its  course; 
2.  upon  the  absence  of  a  sensory  disturbance  noted  evenly  and  constantly 
at  a  certain  level,  and  3.  upon  the  addition  later  of  other  symptoms  charac- 
teristic of  sclerosis,  like  nystagmus,  intention  tremor,  changes  in  the  background 
of  the  eye.  At  any  rate,  as  some  experiences  have  shown,  a  relatively  long 
period  of  observation  may  occasionally  be  necessary,  to  exclude  a  sclerosis 
with  certainty. 

Differentiation  from  a  meningo-myelitis  luetica  may  not  be  easy  at  the 


Fig.  85. — Intra-medullary  spindle-cell  sar- 
coma.    {After  Gowers.) 


DIAGNOSIS  OF  TUMORS  OF  THE  SPINAL  CORD  387 

beginning,  if  relatively  violent  pains  are  caused  by  root  irritation.  But 
lues  spinalis  is  characterized  by  the  fact,  that  no  regular,  absolutely  steady 
advance  of  all  symptoms  can  be  observed  as  in  the  case  of  tumor,  but  that 
in  this  disease  there  is  frequently  considerable  alteration  in  the  violence  and 
extent  of  the  motor  and  sensory  phenomena  of  paralysis;  furthermore,  the 
syphilis  is  not  as  a  rule  confined  to  the  spinal  cord,  so  that  the  diagnosis  is 
made  easier  by  simultaneous  cerebral  symptoms.  In  a  doubtful  case,  the 
employment  of  specific  treatment  will  be  necessary  as  a  further  means  of 
assistance. 

We  face  a  far  more  difficult  problem,  if  it  be  a  question  of  the  practically 
so  important  differentiation,  whether  we  are  dealing  with  a  tumor  of  the 
spinal  membranes  or  with  a  compression  paralysis  from  another  cause; 
through  intra-medullary  or  vertebral  tumors,  spondylitis  tuberculosa,  pachy- 
meningitis cervicalis  hypertrophica  or  a  circumscribed  meningitis  serosa 
spinalis  {Oppenheim  and  Krause). 

1.  The  determination  of  a  caries  and  a  vertebral  tumor  is  very  frequently 
made  very  difficult,  or  even  quite  impossible,  by  the  fact  that  in  the  beginning 
of  the  disease,  even  with  the  most  minute  examination  of  the  spinal  column, 
no  deformity  or  localized  tenderness  can  be  demonstrated  and  that  examina- 
tion with  the  X-rays  has  had  a  negative  result.  A  greater  degree  of  stiffness 
of  the  spinal  column  and  painfulness  upon  motion  of  the  trunk  will  certainly, 
even  without  externally  noticeable  change  in  form,  count  in  favor  of  a  devel- 
oping vertebral  affection,  since  even  large  tumors  of  the  membranes,  espe- 
cially in  their  favorite  location  in  the  dorsal  segment,  exist,  as  a  rule,  without 
considerable  disturbances  in  movement.  Besides  this  in  doubtful  cases, 
the  detection  of  signs  of  recent  or  arrested  tuberculosis,  of  fever,  or  gravitation 
abscesses  and  where  a  carcinoma  is  suspected,  the  discovery  of  a  malignant 
tumor  in  other  organs,  or  the  existence  of  a  marked  amount  of  cachexia,  are 
important  points  for  diagnostic  purposes. 

2.  Concerning  differentiation  from  a  pachymeningitis  cervicalis,  it  is 
to  be  remembered  that  this  disease  is  located  preferably  in  the  cervical  seg- 
ment, while  the  majority  of  tumors  are  to  be  found  in  and  opposite  the  dorsal 
segment.  Furthermore,  the  disease  progresses  far  less  rapidly  than  a  tumor 
of  the  medulla  spinalis  and  develops  preferably  in  individuals  who  formerly 
had  syphilis;  in  most  instances  it  exists  for  years  and  decades  until  more 
serious  disturbances  in  cord  conduction  are  developed. 

3.  The  question,  whether  an  intra-medullary  tumor  is  present,  or  one 
emanating  from  the  meninges  can,  in  many  cases,  not  be  answered  with 
certainty.  A  pronounced  neuralgic  prodromal  stage  presupposes  in  the  first 
instance  an  extra-medullary  location.  For  even  if  the  spinal  cord  tumors 
may  be  accompanied  by  pains,  due  to  concomitant  chronic  meningitis  and 
to  irritation  of  intra-spinal  tracts  sensitive  to  pain,  nevertheless  it  is  usually 
a  matter  of  only  transitory  and  slightly  pronounced  sensory  symptoms  of 


388  DISEASES  OF  THE  SPINAL  CORD 

irritation.  But  unfortunately,  in  recent  times,  observations  of  meningeal 
tumors  with  painless  course  have  become  more  frequent,  so  that  the  absence 
of  a  neuralgic  stage  by  no  means  excludes  an  extra-medullary  seat.  The 
experience  mentioned  above,  is  important,  viz.,  that  the  neoplasms  originating 
in  the  cord,  especially  if  a  glioma  is  connected  with  formation  of  cavities, 
often  evoke  in  the  beginning  of  the  disease  partial  sensory  paralysis.  A 
further  diagnostically  important  sign  of  intra-medullary  tumor  consists,  as 
has  already  been  mentioned,  in  the  fact  that  they  not  infrequently  display  a 
rapid  growth  upwards  and  downwards  and  thereby  simulate  the  picture  of  a 
subacute  ascending  myelitis,  whereas  for  the  meningeal  tumors,  it  is  the 
slight  displacement  of  the  upper  boundary  of  the  sensory  disturbance,  in 
spite  of  increase  of  the  symptoms  of  paralysis,  that  is  characteristic. 

4.  The  syringomyelias  may  be  distinguished  from  the  meningeal  tumors 
mostly  by  the  fact,  that  a  real  neuralgic  stage  is  always  absent  in  them,  even 
though  slight  pains  and  paraesthesias  form  no  unusual  concomitant  phenom- 
enon; that  furthermore,  the  sensory  disturbance  bears  a  typically  dissociated 
character  and  is  confined  to  a  cutaneous  region,  supplied  by  a  definite  segment 
of  the  spinal  cord,  while  in  a  pressure  paralysis  of  the  cord,  by  interruption 
in,  the  conductivity  of  the  sensory  tracts,  sensory  paralyses  of  one  or  both  sides 
appear  below  the  point  of  compression.  To  this  must  be  added  the  further 
fact  that  syringomyelia  progresses  more  quickly  upwards,  possibly  even  into 
the  medulla  oblongata,  and  only  after  a  much  longer  period  and  in  long- 
continuing  stationary  intervals  gives  rise  to  such  severe  paralyses  of  the 
lower  extremities  as  are  usually  developed  in  unbroken  succession  in 
meningeal  tumors  as  soon  as  the  first  signs  of  a  compression  of  the  cord 
have  appeared. 

5.  For  the  differential  diagnosis  between  extra-medullary  tumors  and 
that  form  of  a  localized  serous  meningitis,  which  has  been  described  by 
Oppenheim  and  Krause  and  which  can  imitate  in  a  very  deceptive  manner  the 
disease  picture  of  a  tumor,  we  lack  at  present  any  secure  foundation. 

Determination  of  the  Location  of  the  Tumor. — The  exact  determina- 
tion of  the  height  at  which  the  tumor  is  located  in  the  spinal  cord  is  of  the 
greatest  practical  importance,  since  upon  it,  in  the  first  place,  depends  the 
success  of  operative  interference. 

.  As  an  important  indication  for  determining  the  location,  an  occasionally 
demonstrable  locally  circumscribed  vertebral  tenderness  may  be  serviceable. 
This  will  be  especially  important,  if,  at  the  same  time,  neuralgias  appear  in 
a  root  area,  the  appertaining  spinal-cord  segment  of  which,  according  to  the 
knowledge  we  now  possess,  lies  opposite  to  the  vertebra  that  is  sensitive  to 
pressure.  Tenderness  to  pressure,  symptoms  of  root  irritation  and  of  functional 
loss,  however,  must  be  in  harmony  with  one  another,  since  the  sensitiveness 
of  the  spinal  column  as  a  result  of  chronic  meningitis  or  for  other  unknown 
reasons,  is  frequently  far  more  extended  than  would  correspond  to  the  size 


LOCALIZATION  OF  TUMORS  OF  THE  SPINAL  CORD         389 

of  the  tumor  and  an  isolated  area  of  pain  on  pressure  can  occasionally 
develop  on  a  purely  nervous  foundation. 

In  the  majority  of  cases,  local  tenderness  to  pressure  is  not  demonstrable, 
and  even  pains  may  be  entirely  wanting,  so  that  we  are  limited  to  the  sensory 
and  motor  symptoms  of  loss  of  function. 

The  possibility  of  establishing  the  location  of  a  tumor  in  a  certain  segment 
of  the  spinal  cord,  on  the  basis  of  sensory  phenomena  of  functional  loss, 
rests  upon  our  knowledge  of  the  relations  of  cutaneous  sensibility  to  the  indi- 
vidual segments  of  the  spinal  cord.  Each  of  these,  namely,  receives  the  majority 
of  the  sensory  fibres  of  a  definite  rather  sharply  defined  cutaneous  region. 
If,  therefore,  a  single  segment  is  injured  by  the  presence  of  a  tumor,  a  dis- 
turbance in  sensation  makes  itself  felt,  as  a  rule,  in  the  corresponding  region 
of  the  skin.  As  may  be  seen  from  the  scheme  of  Edinger  presented  herewith 
(Fig.  86)  the  cutaneous  regions  belonging  to  the  dorsal  segments  of  the 
spinal  cord  form  stripes  that  course  horizontally  upon  the  skin  of  the  trunk,  so 
that  after  the  destruction  of  a  dorsal  segment  a  horizontal  boundary  of 
sensory  disturbances  appears  upon  the  trunk. 

Clinical  observation  has  taught  us,  furthermore,  that  after  injury  of  a 
single  segment,  no  complete  loss  of  sensation,  but  only  a  lowering,  a  hypces- 
thesia,  appears  in  the  appertaining  stripe  upon  the  skin.  This  rests  on  the 
fact  that  every  individual  cutaneous  region  sends  its  nerve  fibres  to  two  or 
three  segments  placed  one  above  the  other,  so  that  several  of  them  must  be  injured 
by  a  tumor,  if  a  total  anasthesia  is  to  be  found  in  a  definite  cutaneous  area. 
This  condition,  that  the  lesion  of  a  single  segment  of  the  spinal  cord  causes  in 
the  appertaining  cutaneous  area  only  a  lowering,  but  not  a  total  loss  of  sensi- 
bility, is  of  special  importance  for  the  establishment  of  the  upper  limit  of  a 
tumor. 

If  a  tumor  injures  the  spinal  cord  at  a  certain  level  by  pressure,  there 
occurs  in  the  place  of  strongest  compression  of  the  cord  an  interruption  in  the 
conduction  of  the  ascending  sensory  fibres  of  the  spinal  cord.  As  a  result  of  this, 
there  is  a  general  sensory  paralysis  in  that  entire  part  of  the  body  which  lies 
below  the  point  of  compression.  The  tumor,  however,  causes  also  local  seg- 
mentary disturbances,  since  its  upper  end,  which  generally  extends  above  the 
region  of  the  most  severe  transverse  lesion,  affects  also  the  spinal  cord  seg- 
ments lying  above  this  place,  and  gives  rise  to  disturbances  in  sensibility  in 
their  appertaining  cutaneous  areas. 

The  scheme  of  Fig.  86  serves  best  for  the  explanation  of  these  relations. 
By  the  gross  lines  is  meant  that  in  the  lower  extremities  and  in  the  trunk  up 
to  about  a  hand's  breadth  of  the  sternum,  the  ability  of  perceiving  sensations 
is  completely  lost,  and  that  above  this  region  with  total  anaesthesia  there  is  a 
cutaneous  stripe  (oblique  lines,  uncrossed),  which  shows  only  a  diminution  of 
sensation. 

This  hypasthetic  cutaneous  area,  which  therefore  forms  the  upper  limit  of 


39° 


DISEASES  OF  THE  SPINAL  CORD 


Fig.  86. — Disturbance  of  sensibility  in  myelitis  due  to  compression  (schematic).  The  dis- 
turbance in  sensibility  reaches  in  the  trunk  the  cutaneous  region  of  the  7th  dorsal  segment. 
The  hypothetic  cutaneous  area  is  shown  by  simple  cross  lines.  The  double  cross  lines  show 
the  anaesthetic  area.      (After  Edinger.)  - 


LOCALIZATION'  OF  TUMORS  OF  THE  SPINAL  CORD  391 

the  sensor}T  disturbance,  according  to  Edinger'' s  scheme  belongs  to  the 
seventh  dorsal  segment. 

According  to  the  above  explanation,  we  must  interpret  the  origin  of  the 
sensory  paralysis  thus  schematically  presented  in  this  way,  that  through  the 
pressure  of  a  tumor  an  interruption  in  conduction  of  the  sensory  spinal-cord 
tracts  lying  below  the  seventh  dorsal  segment  resulted,  which  caused  a  total 
anaesthesia  of  that  part  of  the  body  lying  below  the  place  of  compression. 
But  we  must  assume  in  addition,  that  the  upper  end  of  the  tumor  extends 
to  the  seventh  dorsal  segment,  since  the  cutaneous  area  belonging  to  it  still 
exhibits  a  diminution  in  sensibility. 

If  we  review  the  course  of  the  investigation  in  determining  the  location  of 
a  neoplasm,  it  is  in  the  first  place  a  matter  of  the  most  accurate  determination 
that  is  possible  of  the  upper  boundary,  which  shows  barely  demonstrable  sensory 
disturbances.  With  the  aid  of  the  well-known  sensory  area  schemes  of 
Edinger,  Seiffer,  and  others,  we  get  our  bearings  to  which  spinal-cord  segment 
the  cutaneous  area  belongs,  which  according  to  the  result  of  the  investigation 
forms  the  upper  limit  of  the  sensory  disturbance.  If,  e.  g.,  the  upper  limit,  as 
drawn  in  the  figure,  reaches  to  the  height  of  the  seventh  rib  upon  the  trunk, 
according  to  Edinger'' s  scheme,  the  hypothetic  cutaneous  stripe  corresponds 
to  the  seventh  dorsal  segment;  we  may  therefore  expect  the  upper  end  of  the 
tumor  to  be  operated  on  to  be  opposite  Dy.  As  may  also  be  seen  from 
Gower's  scheme  (Fig.  84  on  p.  383),  Dj  (oblique  lines)  lies  opposite  to  the 
sixth  dorsal  vertebra  and  fifth  dorsal  spinal  process.  In  order  to  expose  the 
tumor,  therefore,  the  operator  will  make  his  incision  at  the  level  of  the  fifth 
dorsal  spinous  process  and  remove  the  sixth  and  the  seventh,  possibly  also 
the  fifth  vertebral  arch,  since  experience  has  shown  that  the  seat  of  the  tumor 
is  generally  assumed  too  low  rather  than  too  high.  In  otherwise  correct 
diagnoses  of  the  tumors  in  several  cases  the  neoplasm  was  not  found  because 
of  a  laminectomy  that  was  performed  too  low.  The  longitudinal  extent 
downwards  cannot,  as  a  rule,  be  determined,  especially  when  the  location  of 
the  tumor  is  in  the  dorsal  segment,  since  this  causes  in  addition  to  the  local 
phenomena  of  functional  loss  and  irritation  at  the  upper  boundary,  general 
disturbances  in  conduction  below  the  point  of  compression  due  to  the  pressure 
on  the  entire  transverse  section  of  the  spinal  cord,  which  prevent  us  from 
recognizing    the    segmentary    arrangement    of    the    sensor}*    disturbances. 

For  the  determination  of  the  location  of  the  neoplasms  in  the  cervical  and 
lumbar  segments,  we  must  consider  not  only  the  sensor}*,  but  also  the  motor 
phenomena  of  functional  loss  and  the  disturbances  of  the  reflexes.  The  dis- 
tribution of  the  muscular  innervation  among  the  various  segments  of  the 
spinal  cord  shows  the  same  regularity  as  the  sensory  supply  of  the  skin,  in  as 
much  as  every  muscle  is  subserved  by  several  neighboring  segments.  Our 
knowledge,  which  is  as  yet  incomplete  of  the  segments  belonging  to  the 
individual  muscles  is   summarized,   as  it  were,   in  Edinger s  table.     The 


392  DISEASES  OF  THE  SPINAL  CORD 

localization  diagnosis  at  the  levels  of  the  lumbar  enlargement  and  the  conus 
terminalis  is  particularly  difficult,  because  the  roots  of  this  region  after  their 
exit  from  the  spinal  cord,  descend  for  some  distance  before  they  leave  the 
spinal  canal.  As  Schultze  proved,  in  a  corresponding  localization  of  the 
tumor  in  the  conus  terminalis,  the  same  symptom  complex  may  arise  as  in  a 
compression  in  the  region  of  the  last  lumbar  vertebra,  or  of  the  sacral  vertebrce. 
These  conditions  will  be  discussed  in  more  detail  in  a  separate  chapter  on 
diseases  of  the  conus. 

Treatment. — As  soon  as  a  suspicion  of  spinal  coral  syphilis  is  aroused, 
or  of  tubercular  caries,  which  in  so  many  cases  cannot  be  excluded,  our  first 
task  is  to  order  antisyphilitic  treatment,  or  to  try  treatment  by  extension. 
In  tumor,  after  extension  treatment  of  the  spinal  column,  the  patient  grows 
worse  with  unusual  frequency;  but  this  has  occasionally  been  observed  in 
caries  also,  so  that  no  decisive  importance  can  be  attributed  to  this  phenom- 
enon. If  the  treatment  fails  and  the  disease  progresses,  we  face  the  question 
of  operative  interference,  which  because  of  the  unusually  favorable  results 
that  have  heretofore  been  attained  in  meningo-spinal  tumors,  must  always 
be  taken  into  consideration. 

In  every  case,  in  which  the  typical  symptomatic  picture  of  a  meningo- 
spinal  tumor  is  present,  and  a  determination  of  the  locality  seems  possible, 
surgical  interference  is  earnestly  urged  for  even  if  a  mistake  in  diagnosis 
is  occasionally  unavoidable,  since  the  typical  disease  picture  of  an  operable 
meningo-spinal  tumor  is  simulated  by  vertebral  disease  or  by  a  tumor  of  the 
cord,  nevertheless,  the  chances  of  complete  cure  by  removal  of  the  usually 
benign  meningo-spinal  tumor,  are  quite  favorable. 

We  are  also  absolutely  justified  in  suggesting  to  the  patient  an  explorative 
laminectomy,  even  though  the  atypical  disease  picture  indicates  a  progressive 
compression  of  the  spinal  cord,  and  we  are  unable  even  after  long  observation 
to  make  a  certain  diagnosis  between  intra-  and  extra-medullary  tumors. 
As  long  as  in  the  progressive  course  of  the  disease,  a  meningo-spinal  tumor 
cannot  be  excluded,  an  exploratory  opening  of  the  spinal  canal  is  advisable, 
since  without  an  operation  the  patient  is  steadily  progressing  to  certain 
death.     An  operation,  if  the  symptoms  point  to  multiple  tumors,  is  futile. 

Prognosis. — The  chances  of  recovery  are  naturally  most  favorable, 
if  the  operation  is  performed  as  soon  as  possible,  before  Jhe  development  of 
the  most  severe  disturbances  in  conduction.  As  a  rule,  a  diagnosis  of  a 
tumor  and  the  more  exact  determination  of  its  location,  will  be  possible 
only  after  the  development  of  pronounced  phenomena  of  compression  on 
the  part  of  the  cord.  But  even  considerable  disturbances  in  conduction, 
complete  paraplegias  and  bladder  disturbances  may  absolutely  disappear 
in  the  course  of  weeks  or  months,  if  the  pressure  lesion  has  not  lasted  too 
long. 

The  most  favorable  situation  of  tumors  for  an  operation,  is  the  dorsal 


PACHYMENINGITIS  CERVICALIS  HYPERTROPHICA        393 

portion  of  the  spinal  cord,  less  favorable  trie  cervical  and  lumbar  portions, 
as  well  as  the  cauda  equina  region. 


(d)  Pachymeningitis  Cervicalis  Hypertrophica  (Charcot) 

That  form  of  chronic  inflammation  of  the  membranes  of  the  spinal  cord, 
described  by  Charcot  under  this  name  is  a  very  rare  disease.  Anatomically 
the  most  striking  finding  is  a  very  considerable  thickening  of  the  dura  mater, 
which  may  possibly  attain  from  five  to  ten  times  its  normal  size.  The  soft 
membranes  likewise  undergo  inflammatory  changes,  becoming  adherent  to 
each  other  and  to  the  spinal  cord.  The  microscopic  picture  shows  not  only 
dense,  newly  formed  bands  of  connective  tissue,  but  also  regularly  a  consider- 
able nuclear  infiltration  and  a  thickening  of  the  vessels  of  the  spinal  cord  as 
well  as  of  its  membranes.  The  inflammatory  process  does  not,  in  all  prob- 
ability, begin  always  in  the  dura,  but  originates  also  in  the  soft  membranes; 
it  is  usually  most  marked  in  the  cervical  portion;  frequently,  however,  it 
extends  upwards  to  the  meninges  of  the  brain  and  downwards  to  the  lumbar 
segment  of  the  spinal  cord. 

By  mechanical  compression  and  extension  of  the  inflammatory  process, 
the  roots  and  the  cord  itself,  the  latter  at  first  especially  in  its  marginal  por- 
tions, become  injured.  But  also  more  extended  degenerations  of  the  nerve 
fibres  with  chronic  inflammatory  changes  of  the  cord  and  secondary  ascend- 
ing and  descending  degenerations  may  develop. 

Of  its  cetiology  we  know  only  with  certainty,  that  the  disease  is  very 
frequently  preceded  by  a  syphilitic  infection,  while  the  importance  of  the 
other  aetiological  factors  held  responsible,  like  alcoholism,  colds,  over-exer- 
tion, is  quite  uncertain. 

Symptomatology. — The  first  phenomena  of  the  disease,  which  may 
constitute  the  whole  clinical  picture  for  several  months,  consist  of  pains 
beginning  in  the  back  of  the  neck  and  radiating  thence  to  the  back  of  the  head, 
the  shoulders  and  arms.  These  spontaneously  appearing  pains,  increasing 
upon  pressure  and  motion,  are  referred  preferably  to  the  region  of  the  ulnar 
and  the  median  nerves.  To  these  phenomena  of  irritation,  based  on  com- 
pression of  the  posterior  roots,  there  are  added  later  objective  sensory  dis- 
turbances, likewise  paralysis  and  atrophy  in  the  muscles  of  the  arm  and  hand, 
due  to  degeneration  in  the  anterior  motor  roots.  Through  the  fact,  that  the 
radial  region  is  frequently  spared  for  some  time,  a  peculiar  position  of  the 
hand  and  fingers  develops,  due  to  the  overbalance  of  the  extensors  of  the 
hand  and  fingers:  dorsal  flexion  of  the  entire  hand  and  extension  of  the 
basal  phalanges,  while  the  middle  and  terminal  phalanges  are  flexed 
(preacher's  hand).  During  the  advance  of  the  process,  there  are  added, 
sooner  or  later,  to  the  root  symptoms,  signs  of  a  compression  of  the  cord: 
slowly  increasing  paresis  of  the  legs  with  considerable  exaggeration  of  the 


394  DISEASES  OF  THE  SPINAL  CORD 

tendon  reflexes,  mostly  slight  sensory  disturbances  in  the  lower  extremities, 
disturbances  in  functions  in  the  bladder  and  rectum. 

Course  and  Prognosis. — The  disease,  as  a  rule,  extends  over  many 
(15  to  20)  years.  Death  may  result  from  decubitus  or  cystitis.  Improve- 
ment and  standstill  are  observed  in  every  stage. 

Diagnosis. — Differentiation  of  the  disease,  is  as  a  rule  impossible  at 
the  beginning,  since  the  compression  paralyses  due  to  meningo-spinal  tumors 
and  tumors  of  the  spinal  column  as  well  as  to  tubercular  caries,  may,  at  first, 
evoke  similar  symptoms  of  root  irritation,  without  the  possibility  of  always 
demonstrating  the  early  vertebral  changes.  Only  the  course  that  drags  on 
for  years  and  the  simultaneous  absence  of  deformities  in  the  spinal  column 
will  point  the  way  to  the  diagnosis. 

Treatment. — Antisyphilitic  treatment  is  of  chief  importance:  iodide  of 
potassium  for  some  time  (10  gr.  three  times  a  day)  and  mercurial  treatment. 
As  a  derivative  in  more  violent  pains,  the  painting  of  the  region  of  the  neck 
with  iodine  and  ferrum  candens  may  be  recommended  in  particular,  in 
addition  to  medication  with  anti-neuralgics. 

III.  The  Syphilitic  Diseases  of  the  Spinal  Cord 

Syphilis  as  an  aetiological  factor  plays  an  extraordinarily  important  role 
in  the  diseases  of  the  spinal  cord.  We  leave  out  of  consideration,  here, 
such  diseases  of  the  spinal  cord  as  in  individuals  formerly  infected  with 
syphilis  usually  develop  slowly,  in  the  form  of  primary  degenerations  of  definite 
spinal-cord  tracts  and  systems  of  fibres  and  are  denominated  metasyphilitic 
diseases  (tabes  dorsalis).  Here  we  are  concerned  only  with  those  disease 
pictures,  in  which  the  pathologic  anatomic  changes  bear  a  specifically 
syphilitic  character,  or  in  which,  owing  to  a  series  of  concomitant  conditions, 
the  assumption,  that  the  diffuse  changes  in  the  spinal  cord,  which  are  at  the 
basis  of  the  disease,  rest  upon  syphilis,  is  made  highly  probable,  in  spite  of 
the  fact  that  the  anatomic  signs  that  characterize  the  disease  are  not,  in 
every  case,  demonstrable.  The  most  frequent  and  important  form  of  spinal- 
cord  syphilis  is 

Meningomyelitis  Chronica  Syphilitica 

Anatomical  Findings. — The  process  begins  in  the  soft  membranes  of 
the  spinal  cord,  less  frequently  does  it  arise  from  the  inner  surface  of  the  dura; 
the  membranes  are  thickened  over  a  large  area  and  permeated  in  a  diffuse 
manner  with  a  dirty  and  jelly-like  gummatous  granulation-tissue.  Circum- 
scribed gummatous  tumors  are  rare.  Beginning  from  the  gummatous  new 
growth,  which  surrounds  the  spinal  cord  like  a  rind,  prong-like  cellular 
proliferations  are  seen  advancing  into  the  marginal  portions  and  extend  even 


CHRONIC  SYPHILITIC  MENINGOMYELITIS 


395 


deeper  into  the  gray  matter  of  the  cord  (Fig.  88) ;  the  roots  of  the  spinal  cord 
likewise  usually  exhibit  signs  of  a  considerable  infiltration  with  round  cells. 
Through  the  granulation  tissue,  which  enters  the  substance  of  the  cord  like 
a  wedge  and  through  the  frequent  and  usually  simultaneously  appearing 
end-arteritic  changes  of  the  vessels  of  the  spinal 
cord,  the  disease  is  spread  to  nerve  tissue.  Due  to 
the  pressure  of  the  disease  foci  advancing  from  the 
periphery  in  the  form  of  prongs  an  atrophy  of  the 
nerve  fibres  results.  To  this  is  added  as  further  dis- 
turbance the  deficient  blood  supply  of  the  substance 
of  the  spinal  cord  through  the  diseased  vessels,  the 
thickening  of  whose  walls  may  bring  about  a  com- 
plete closure  of  the  lumen  of  the  vessel ;  in  addition 
to  atrophy  of  nerve  fibres,  there  are  found  ischemic  softenings  of  the  cord 
substance,  small  hemorrhages  and  occasionally  even  cavity  formations.  The 
gummatous  meningitis  in  most  cases  is  most  strongly  developed  at  the  pos- 
terior part  of  the  spinal  cord.  If  the  process,  as  has  been  observed  in  some 
cases,  remains  limited  to  a  marked  degree  to  the  roots  of  the  spinal  cord,  so 
that  many  of  them  appear  thickened  like  knots  and  swollen,  the  condition 


Fig.  87. — Multiple  root 
neuritis  in  syphilis.  (After 
Butter  sack.) 


Fig.  88. — Meningomyelitis  syphilitica.  Penetration  of  the  cord  by  the  new  growth. 
«,  New  growths;  w,  roots  of  the  spinal  cord  imbedded  in  the  gummatous  layers.  (After 
Boettiger.) 


is  spoken  of  as  a  multiple  radicular  neuritis  (Fig.  87).     All  the  changes  here 
described  may  be  found  also  in  congenital  syphilis  of  the  spinal  cord. 

Symptomatology.— It  is  easily  understood,  that  with  the  variety  of 
anatomical  changes  caused  by  syphilis,  the  disease  picture  may  be  a  very 
variable  one,  differing  according  to  the  localization  and  the  predominance 
of  lesions  of  the  nerve  tissue  as  caused  either  by  arteritic  processes,  by 
gummatous  meningitis  or  by  radicular  neuritis.  But  Oppenheim  has  demon- 
strated that  a  symptom  complex  may  be  mapped  out  for  the  syphilitic  form 
of  meningomyelitis  which,  because  of  the  simultaneous  appearance  of  men- 


396  DISEASES  OF  THE  SPINAL  CORD 

ingeal,  radicular  and  cord  symptoms  as  well  as  through  certain  peculiarities 
of  the  course,  bears  an  imprint  so  characteristic,  that  the  diagnosis  in  many 
cases  may  be  made  quite  easily. 

Besides  local  pains  in  the  back  and  in  the  sacral  region  there  appear 
radiating  pains  due  to  root-irritation,  which,  according  to  the  location  of  the 
disease,  are  described  as  "girdle-feeling"  or  as  neuralgias  in  the  extremities. 
Since  first  and  predominantly  the  posterior  half  of  the  spinal  cord  is  affected, 
motor-root  symptoms  appear,  as  a  rule,  if  at  all,  only  later  and  then  in  the 
form  of  partial  atrophic  paralyses  in  the  arms  and  legs.  To  these  meningeal 
and  radicular  irritation  phenomena  are  added  the  important  symptoms  of  a 
lesion  of  the  cord:  unilateral  or  bilateral  spastic  paresis  of  the  legs  caused  by 
the  affection  of  the  dorsal  portion  of  the  cord.  If  the  cervical  portion  is 
affected  in  its  enlargement,  the  paralysis  of  the  arms  is  flaccid  and  atrophic, 
and  is  associated  with  spastic  phenomena  in  the  legs,  while,  if  the  lesion  is 
located  in  the  lumbar  portion,  the  paralysis — restricted  to  the  lower  extrem- 
ities— is  flaccid,  from  the  first.  The  development  of  the  disturbances  in 
motility  may  develop  gradually  or  quite  suddenly;  likewise,  at  any  time,  a 
slight  paraparesis  may  quickly  change  into  a  total  paraplegia.  Objective 
disturbances  in  sensation  and  in  the  functions  of  the  bladder  and  rectum  are 
found  regularly.  Quite  frequently  the  picture  of  a  Brown-Sequard  paralysis 
appears  temporarily  as  an  effect  of  a  more  unilateral  compression.  If,  at 
one  of  the  stages  of  the  development  of  the  disease,  the  posterior  roots  are 
more  extensively  and  more  markedly  affected  by  the  gummatous  changes,  a 
disease  picture  may  occasionally  appear  showing  absence  of  the  patellar  reflex, 
lancinating  pains,  sensory  disturbances,  which  closely  resembles  a  tabes 
dorsalis,  especially  if  to  the  spinal  phenomena,  there  are  added  cerebral 
symptoms  analogous  to  those  of  tabes — pupillary  rigidity,  paralysis  of  the 
muscles  of  the  eye — as  a  result  of  basal  meningitis  (Oppenheim) .  Such  cases 
have  been  spoken  of  as  a  pseudotabes  syphilitica. 

The  special  peculiarity,  which  the  course  of  a  meningomyelitis  syphilitica 
is  constantly  showing,  is,  that  frequently,  a  very  striking  change  in  the  inten- 
sity and  extent  of  the  symptoms  just  described  may  be  observed.  A  repeated 
appearance  and  disappearance,  now  sudden,  now -gradual,  of  single  paralytic 
phenomena  is  no  unusual  occurrence.  One  sees  abducens  and  oculomotor 
pareses  disappear  again  after  existing  but  a  few  days,  slight  parapareses  of 
the  legs  quickly  being  transformed  into  severe  paraplegias;  pronounced 
bladder  disturbances  may  disappear  again  in  a  short  time;  in  like  manner 
the  neuralgic  troubles  caused  by  radicular  irritation  show  considerable 
fluctuations  in  severity  and  extent.  Oppenheim  has  called  attention  in 
particular  also  to  the  condition  of  the  tendon  reflexes,  which  may  appear 
diminished,  abolished,  or  actively  increased,  all  within  a  few  days.  It 
has  been  attempted  to  explain  this  change  in  individual  symptoms,  which 
is  occasionally  sudden  and  always  characteristic  of  lues  spinalis  by  the  fact 


ERB'S  SYPHILITIC  SPINAL  PARALYSIS  397 

that,  as  a  result  of  the  change  in  the  amount  of  blood  the  tissues,  the  granula- 
tion tissue,  the  roots  of  the  spinal  cord  and  the  cord  are  exposed  to  pressure 
of  varying  force.  Another  important  sign  of  lues  spinalis  depends  upon 
the  fact  that  the  syphilitic  process  does  not,  as  a  rule,  confine  itself  to  the 
spinal  cord,  but  attacks  also  the  meninges  and  the  vessels  of  the  brain  and 
above  all,  basal  cerebral  phenomena  appear  at  an  early  period.  In  the 
majority  of  cases,  therefore,  there  will  appear,  in  addition  to  the  spinal 
symptoms,  also  some  few  cerebral  paralytic  symptoms — disturbances  in  the 
muscles  of  the  eye  or  in  vision — which  exhibit  the  same  instability  as  do  those 
of  the  spinal  cord. 

In  the  year  1892  Erb  separated  another  disease  entity,  under  the  desig- 
nation of  syphilitic  spinal  paralysis,  from  the  large  and  varied  group  of 
affections  of  the  spinal  cord,  which  are  based  upon  syphilis.  He  had  sus- 
pected, that  similar  anatomic  changes  would  be  found  at  the  basis  of  the 
symptom  complex  sketched  by  him,  as  those  of  the  meningomyelitis  just 
discussed,  that  is,  in  particular,  specific  myelitis,  or  arteritic  and  gummatous 
processes.  This  hypothesis  has  not  found  support  in  the  postmortem  find- 
ings that  are  available  thus  far.  For  it  has  been  shown  that  various  changes 
in  the  spinal  cord  come  under  consideration  as  the  pathologic-anatomical 
foundation  of  ErVs  symptomatic  picture.  In  a  number  of  cases,  a  sclerosis 
of  certain  tracts  of  the  spinal  cord,  of  the  pyramidal  and  lateral  cerebellar 
tracts  and  of  the  columns  of  Goll  were  found,  without  any  participation  of 
the  meninges,  or  any  specific  changes  in  the  vessels  being  noted,  so  that 
anatomically,  the  picture  of  a  primary  combined  system  disease  was  present. 
In  other  cases,  however,  a  chronic,  disseminated  or  partial  myelitis  transversa 
was  found  with  ascending  and  descending  secondary  degenerations,  or  even 
a  combination  of  diffuse  chronic  myelitis  with  apparently  primary  columnar 
degenerations.  The  thickening  of  the  walls  of  the  vessels,  which  was 
demonstrated  especially  in  the  chronic  myelitic  foci,  did  not  usually  exhibit 
the  picture  of  specific  end-arteritic  changes,  even  though  some  observers, 
like  Nonne,  proved  the  presence  of  apparently  specific  end-arteritis  in  some' 
arteries,  in  addition  to  these  ordinary  thickenings  in  the  walls  of  the  vessels. 

Erb's  disease  picture,  in  its  pure  form,  is  rather  clearly  defined.  In 
individuals,  who  previously  have  been  infected  with  syphilis,  there  develops 
gradually,  and  usually  in  an  insidious  manner,  more  commonly  in  the 
earlier  stages  of  syphilis,  about  two  to  six  years  after  the  infection,  a  spastic 
paresis  of  the  legs  with  considerable  increase  of  the  tendon  reflexes  and  the 
Babinski  reflex.  The  gait  is  distinctly  spastic,  even  if  the  muscular  rigidity 
is  but  slightly  pronounced.  Besides  the  weakness  in  the  lower  limbs, 
there  appears  as  a  regular  and  early  symptom,  which  sometimes  precedes 
all  the  other  phenomena,  a  disturbance  of  the  bladder.  The  sensory  disturb- 
ances and  parcesthesias,  which  are  demonstrable  with  equal  frequency,  are 
usually  of  slight  degree.     Variations  from  this  symptomatic  picture  may 


398  DISEASES  OF  TEE  SPINAL  CORD 

occur  as  follows:  the  beginning  of  the  disease  may  occasionally  be  rather 
acute  with  quickly  developing  paraplegia  and  muscular  contractures.  In 
the  pure  form  of  syphilitic  spinal  paralysis,  according  to  Erb,  we  always 
note  the  absence  of  more  severe  pains,  of  more  severe  sensory  disturbances,  as 
well  as  of  all  phenomena  which  point  to  a  disease  of  the  brain  or  its  membranes. 

The  course  of  the  disease  is,  as  a  rule,  slow,  extending  over  many  years, 
with  occasional  improvements  and  standstills.  But  there  is  also  a  form 
with  a  more  rapid  course  and  a  fatal  termination  after  a  few  years. 

Diagnosis. — Considering  the  frequency  of  spinal  diseases  originating 
upon  a  syphilitic  foundation,  and  the  ability  of  syphilis  to  appear  through 
its  multiple  distribution  in  all  the  different  parts  of  the  nervous  system  in 
the  most  varied  symptomatic  pictures,  it  would  be  well  always  to  think  of 
syphilis  in  all  diffuse  diseases  of  the  spinal  cord,  unless  we  have  before  us 
absolutely  typical  disease  pictures. 

In  our  inquiry  into  the  previous  history  and  our  investigation  of  all  the 
other  organs  of  the  body,  we  must  make  it  our  chief  aim  to  look  up  former 
diseases,  in  which  there  was  a  suspicion  of  syphilis,  and  notice  with  special 
care  all  signs  of  recent  or  past  lues.  According  to  experience,  however,  our 
endeavors  in  this  direction  are  not  rarely  entirely  fruitless,  even  where  the 
disease  of  the  spinal  cord  proves  later  to  be  surely  syphilitic. 

Differentiation  of  a  meningomyelitis  syphilitica  from  a  simple  acute  or 
subacute  transverse  myelitis  and  from  a  compression  paralysis  of  the  spinal 
cord,  will  as  a  rule,  cause  no  difficulties.  The  more  severe  pains  and  a 
neuralgic  prodromal  stage  do  not  belong  to  the  disease  picture  of  an  acute 
myelitis.  As  contrasted  with  a  caries,  which  exists  for  a  long  time  without 
deformity  of  the  spinal  column,  sufficient  signs  for  distinction  will  be  found 
in  the  fact  that  the  compression  paralysis,  as  a  rule,  has  a  gradual  steady 
course,  whereas  the  lues  spinalis  is  characterized  particularly  by  the  coming 
and  going  of  phenomena  and  usually  creates  a  disease  picture  pointing  to 
multiple  foci.  The  distinction  from  multiple  sclerosis,  which  likewise  fre- 
quently exhibits  a  striking  change  of  sudden  deteriorations  and  improve- 
ments, can  occasionally  cause  difficulties.  The  differential  diagnosis  will 
mostly  be  based  on  the  fact,  that  pronounced  sensory  phenomena  of  irrita- 
tion are  absent  in  sclerosis  and  that,  on  the  other  hand,  the  characteristic 
intention  tremor,  the  speech  disturbance,  the  partial  optic  atrophy  and  nystag- 
mus are  foreign  to  lues  spinalis. 

Both  multiple  sarcomatosis  and  carcinomatosis  of  the  membranes  may  give 
rise  to  a  confusion  with  lues  spinalis.  Here  only  the  further  course  and  the 
result  of  specific  treatment  will  make  diagnosis  possible. 

ErVs  syphilitic  spinal  paralysis,  in  its  pure  form,  presents  an  easily  defined 
disease  picture;  it  is  distinguished  from  the  simple  spastic  spinal  paralysis 
{Erb)  by  the  presence  of  sensory  and  bladder  disturbances,  from  meningo- 
myelitis by  the  absence  of  sensory  meningeal  and  radicular  irritation  symp- 


PROGNOSIS  AND  TREATMENT  OF  SPINAL  SYPHILIS        399 

toms  and  the  absence  of  cerebral  phenomena.  In  differentiation  from 
combined  disease  of  the  posterior  and  lateral  columns,  the  course  of  the 
disease  must  be  specially  considered.  In  combined  columnar  disease,  there 
is  a  steadily  progressing  process,  which  step  by  step,  attacks  one  system  after 
the  other.  The  course  of  Erb's  disease  is  not,  on  the  other  hand,  so  regular; 
standstills  and  improvements  are  not  unusual;  likewise  even  total  disappear- 
ance of  single  symptoms  under  specific  treatment. 

Erb  himself  emphasized  the  fact,  that  a  large  number  of  transitional 
forms  with  their  deviations,  which  are,  in  part,  considerable,  from  the  pure 
picture  of  syphilitic  spinal  paralysis,  may  often  exhibit  far-reaching  resem- 
blance to  the  most  varied  forms  of  lues  spinalis,  so  that  certain  differentia- 
tion, particularly  from  meningomyelitis,  is  frequently  impossible.  Many 
prominent  neurologists  advocate  the  point  of  view  {Oppenheim  and  others) 
that  the  symptom  complex  described  by  Erb  represents  only  an  initial  stage, 
or  the  picture  of  one  phase  of  meningomyelitis  chronica  syphilitica. 

Prognosis. — Complete  recovery  from  lues  spinalis  is  possible;  it  is  to  be 
expected  particularly  when  the  disease  is  treated  early,  before  the  phenomena 
of  a  lesion  of  the  cord  have  appeared.  But  even  after  the  development  of 
an  incomplete  transverse  lesion,  there  is  still,  according  to  Oppenheim' s 
experience,  possibility  of  absolute  restoration.  In  the  great  majority  of 
cases,  however,  the  outcome  of  the  disease,  as  soon  as  the  cord  itself  has 
suffered,  is  quoad  restiturionem,  unfavorable,  as  more  or  less  considerable 
functional  disturbances  remain  in  the  form  of  spastic  pareses,  bladder  dis- 
turbances, etc.  The  prognosis  even  in  the  cases  whose  course  is  favorable 
is,  nevertheless,  made  less  favorable  by  the  fact  that  a  relapse  may  occur  at 
any  time. 

Treatment. — The  treatment  consists  in  inunction  which  is  continued 
for  several  weeks  (using  1-2  drams  ung.  hydrarg.)  with  simultaneous 
administration  of  iodide  of  potassium  (at  the  beginning  15  grs. ;  later, 
60  grs.  daily) .  Oppenheim  recommends  a  rapid  increase  in  the  dosage  of 
iodide  of  potassium,  or  to  give  large  doses  at  once  (up  to  200  and  300  grs.  pro 
die) ;  he  has  also  had  results  from  internal  or  subcutaneous  employment  of 
iodipin  (10-20  g.  of  the  25%  solution  injected  daily  for  8-10  days),  when 
mercury  and  iodide  of  potassium  remained  ineffective. 

The  inunctions  of  1  to  2  drams  ung.  hydrarg.  pro  die,  had  better  be  made 
in  the  evening  before  going  to  bed,  since  the  absorption  of  the  mercury  is 
effected  in  the  inunction  treatment,  mainly  through  inhalation  of  the  mercu- 
rial fumes.  The  most  suitable  places  upon  the  body  to  be  used  alternately, 
have  been  shown  to  be  the  calves,  the  outer  and  posterior  surface  of  the  thighs, 
the  region  of  the  abdomen  and  the  loins,  the  lateral  parts  of  the  thorax,  the  outer 
and  posterior  surface  of  the  fore-  and  upper  arm.  According  to  experience, 
the  following  parts  have  proved  sensitive  to  the  salve,  the  flexor  aspect  of 


4oo  DISEASES  OF  THE  SPINAL  CORD 

the  joints,  the  axilla,  the  inner  surface  of  the  thighs  and  all  places  upon  the 
skin  with  but  a  small  amount  of  underlying  softer  parts  as,  e.  g.,  the  skin  over 
the  tibias.  Between  each  group  of  five  days,  on  which  inunctions  but  no 
baths  are  given,  one  sandwiches  in  a  sixth  day,  in  which  the  patient  is 
treated  only  to  a  warm  cleansing  bath.  To  avoid  a  mercurial  disturbance 
of  the  mucous  membranes  of  the  mouth,  which  in  its  initial  stage,  manifests 
itself  by  an  increase  in  the  secretion  of  saliva,  a  metallic  taste  in  the  mouth 
and  a  feeling  as  if  the  teeth  are  growing  longer,  persistent  care  of  the  mouth 
is  essential.     The  mouth  should  be  cleansed  after  every  meal. 

A  repetition  of  the  inunction  treatment  is  necessary,  as  soon  as  new 
symptoms  of  disease  appear.  The  question,  how  long  shall  the  treatment 
be  continued,  Gowers  answers  by  stating  that  after  a  course  of  treatment 
extending  over  6  to  10  weeks  without  visible  results,  further  continuation 
for  the  time  at  least  is  useless.  If  there  has  been  improvement  in  the  dis- 
ease, and  a  standstill  has  been  reached,  it  seems  advisable  to  order  regularly 
in  the  next  few  years,  one  or  two  courses  of  antisyphilitic  treatment,  even  if 
relapses  have  not  occurred.  The  combination  of  the  inunction  treatment 
with  baths  in  various  resorts  (Aachen,  Toelz,  Nenndorf)  is  frequently  recom- 
mended. In  addition  to  the  specific  treatment,  it  is  important  above  all  to 
regulate  the  patient's  entire  manner  of  life :  avoidance  of  all  physical  and 
mental  overe-xertion  and  of  all  sexual  and  alcoholic  excesses. 

IV.  Myelitis 

Definition. — We  are  not  at  present  in  a  position  to  draw  a  sufficiently 
sharp  line  from  the  clinical  or  pathologic-anatomical  point  of  view  between 
disease  pictures,  which  are  based  on  acute  and  subacute  changes  in  the 
spinal  cord  with  the  signs  regarded  as  characteristic  of  an  inflammation, 
and  those,  in  which  phenomena  of  acute  degeneration  and  disintegration  of 
the  cord  substance  are  preponderantly  demonstrable.  Most  clinicians,  there- 
fore, classify  for  the  present  as  myelitis  all  diffuse  and  disseminated  inflam- 
matory and  softening  processes  in  the  spinal  cord,  in  so  far  as  they  are  not 
the  result  of  a  direct  crushing  or  contusion  of  the  cord. 

Classification  of  the  Forms  of  Myelitis  and  Anatomic  Findings 

One  distinguishes  a  transverse  myelitis,  in  which  a  focus,  variable  as  to 
the  height  of  its  location,  takes  up  all  or  the  greater  part  of  the  transverse 
section  of  the  spinal  cord;  also,  a  myelitis  disseminata,  with  numerous  smaller 
and  larger  foci  strewn  over  the  entire  length  of  the  medulla  spinalis,  usually 
associated  with  participation  of  the  medulla  oblongata  and  the  brain;  and 
finally,  poliomyelitis,  which  depends  upon  inflammatory  changes,  localized 
mainly  in  the  anterior  gray  horns. 


MORBID  ANATOMY  OF  MYELITIS  401 

The  more  recent  investigations  have  shown  that  a  separation  of  these 
different  forms  of  myelitis  is  justified  only  in  so  far  as  by  the  nomenclature 
attention  is  called  to  the  chief  focus  of  the  disease  and  the  special  localiza- 
tion of  the  changes  through  which  the  disease  picture  attains  its  peculiar 
stamp.  As  a  rule,  both  in  acute  myelitis  transversa  and  in  poliomyelitis, 
there  are,  in  addition  to  the  main  focus,  countless  small  and  minute  inflam- 
matory foci,  scattered  over  the  gray  and  white  matter,  so  that  in  both  these 
forms  therefore,  there  is  exhibited  the  anatomic  picture  of  a  disseminated 
myelitis. 

A  diffuse  myelitic  focus  may  be  recognized  even  by  palpation  and  by 
macroscopic  inspection,  because  of  its  softer  consistency  and  the  more  vivid 
injection  of  the  vessels  of  the  soft  membranes  in  contrast  to  the  adjacent 
sound  parts  of  the  spinal  cord.  When  the  softening  is  very  pronounced, 
the  grayish-yellow  discolored  substance  of  the  cord  flows  over  the  surface 
of  an  incision,  while  in  less  marked  development  of  the  changes,  only  the 
lines  of  separation  between  the  gray  and  white  matter  seem  blurred.  The 
microscopic  appearance  of  the  hardened  preparation  may  vary  considerably. 
Occasionally  the  inflammatory  changes  of  the  vessels  are  comparatively 
unimportant  as  compared  with  the  acute  phenomena  of  degeneration  of 
the  nerve  elements.  The  axis  cylinders  and  medullated  sheaths  seem  to  be 
much  swollen  and  inflated  like  bubbles,  either  singly,  or  in  groups,  so  that 
the  volume  of  a  nerve  fibre  may  often  amount  to  from  10  to  20  times  its 
normal  size.  Through  disintegration  of  the  diseased  fibres,  there  are 
formed  spaces  in  the  tissue  between  the  nerve  fibres,  which  are  still  un- 
changed; these  spaces  are  filled  with  granular  detritus,  cord  remnants  and 
drops  of  fat  (Fig.  89).  As  the  glia  tissue  also,  at  first  by  swelling,  and 
later  by  disintegration,  participates  in  the  process,  softening  foci  develop. 
Compensation  takes  place  through  the  proliferation  of  the  interstitial  tissue 
in  the  neighborhood  of  the  focus  of  degeneration;  a  sclerotic  scar  tissue  is 
formed.  During  this  process,  the  entire  focus  contains  numerous,  so-called 
granular  cells,  which  are  loaded  with  disintegrated  particles  of  tissue  and 
are  to  be  found  especially  in  the  perivascular  spaces  of  the  vessels,  which 
are  usually  thickened. 

In  some  cases,  especially  in  myelitis  disseminata  and  in  poliomyelitis, 
it  is  not  only  the  phenomena  of  acute  degeneration  in  the  nerve  parenchyma, 
which  occupy  a  prominent  position  in  the  anatomic  picture,  but  also  inflam- 
matory changes  in  the  vessels.  They  are  present  in  the  form  of  pronounced 
dilatation  of  the  arterial  and  venous  pathways,  possibly  with  an  extravasa- 
tion of  blood  into  their  surroundings  and  dense  accumulation  of  round  cells 
in  the  adventitia  of  the  vessels  and  the  perivascular  lymphatic  spaces.  After 
all  more  extensive  processes  of  inflammation  and  softening  in  the  spinal 
cord,  there  is  a  development  of  " secondary  degenerations"  of  the  ascending 
and  descending  spinal-cord  tracts,  which  have  become  separated  from  their 
26 


402 


DISEASES  OF  THE  SPINAL  CORD 


ganglion  cells.  The  soft  membranes  of  the  spinal  cord  are,  as  a  rule,  also 
affected  in  myelitis.  Even  in  acute  poliomyelitis,  in  recent  cases,  changes 
in  the  pia  were  found  by  Dauber,  Schultze,  and  others  in  addition  to  the 
changes  in  the  vessels. 

Bacterial  Findings. — The  presence  of  bacteria  in  the  inflammatory  foci, 
the  meninges  and  the  cerebro-spinal  fluid,  has  been  demonstrated,  hereto- 
fore, only  in  rare  cases,  even  when  the  disease  appeared  in  direct  connection 
with  infectious  processes.     In  the  diseased  tissue  of  the  spinal  cord  itself 


f      .    l%vi'~4* 


'■  : 

... 


Fig.  89. — Myelitic  focus  in  acute  myelitis  with  numerous  swollen  axis  cylinders  (a)  and  lacuna 
through,  disintegration  of  nerve  fibres  (&). 

have  been  found  in  addition  to  tubercle  bacilli,  strepto-staphylo-  and  pneumo- 
cocci,  also  the  bacilli  of  anthrax  and  typhoid;  in  the  cerebro-spinal  fluid,  in 
poliomyelitis,  the  presence  of  extra-cellular  diplococci  was  demonstrated  by 
Schultze  and  others,  the  more  exact  determination  of  which  as  meningococci 
by  cultural  proceedings  was  not  possible;  Strilmpell  was  able  to  isolate 
staphylococci  in  the  fluid  of  a  patient  with  myelitis,  which  had  supervened 
in  a  case  of  whitlow. 

According  to  the  experiments  of  Hoche,  Homen  and  others,  the  rarity  of 
bacterial  findings  may  be  due  to  the  fact,  that  the  bacteria  after  their  pene- 
tration into  the  substance  of  the  spinal  cord  and  into  the  cerebro-spinal 
fluid,  quickly  disappear  again. 

iEtiology. — In  the  aetiology  of  acute  myelitis,  in  all  its  different  forms, 
infections  and  intoxications  play  the  most  important  role.  There  is  probably 
no  acute  infectious  disease,  in  the  wake  of  which  the  appearance  of  a  mye- 
litis has  not  been  observed.     I  must  mention  especially  smallpox,  typhoid, 


ETIOLOGY  OF  MYELITIS  403 

diphtheria,  influenza,  erysipelas,  measles,  scarlet  fever,  malaria,  gonorrhea. 
Even  in  connection  with  purulent  processes  in  the  various  organs  of  the  body  : 
in  whitlow,  perityphlitis,  suppuration  in  the  antrum  of  Highmore,  an  acute 
myelitis  is  occasionally  observed.  Furthermore,  in  syphilis  and  tuberculosis, 
acute  myelitis  occurs,  which,  anatomically,  does  not  present  the  specific 
changes  characteristic  of  these  diseases.  For  the  myelitis,  which  occa- 
sionally appears  in  pregnancy  and  in  the  puerperium,  an  infectious  cause  is 
also  assumed.  Since  investigators  have  succeeded  experimentally,  by  the 
introduction  of  toxic  bacterial  products,  in  producing  inflammatory  changes 
of  the  spinal  cord,  which  bear  great  similarity  to  those  in  acute  myelitis,  it  is 
very  probable  that  myelitis  is  caused  not  only  by  the  local  effects  of  the  bac- 
teria themselves,  but  also  by  the  toxins  produced  by  them;  they  may  reach 
the  spinal  cord  by  means  of  the  blood  and  the  lymphatics. 

Catching  cold,  over-exertion,  and  psychic  excitement  were  formerly  thought 
by  many  to  be  the  only  sources  of  certain  cases  of  acute  myelitis ;  this  seems 
doubtful  in  the  light  of  more  recent  experience;  however,  one  must  assign 
to  them  the  importance  of  an  influential  assisting  and  exciting  cause  under 
certain  circumstances. 

After  poisonings  with  chemical  substances  of  the  most  different  varieties 
— CO,  illuminating  gas,  bisulphide  of  carbon,  chloroform,  lead — a  larger 
number  of  cases  of  myelitis  having  a  more  chronic  and  subacute  course 
rather  than  taking  an  acute  form  are  observed;  but  acute  cases  do  occur  and 
especially  after  poisoning  by  smoke  in  firemen,  suddenly  appearing  phenom- 
ena of  spinal  paralysis  are  not  at  all  uncommon. 

Those  diffuse  changes  in  the  spinal  cord,  which  with  numerous  small 
acute  foci  of  degeneration,  develop  upon  the  basis  of  a  cancerous  cachexia  in 
carcinoma,  furthermore,  in  pernicious  ancemia,  leucaemia,  chronic  nephritis, 
diabetes  and  gout,  exhibit  clinically,  as  a  rule,  also  a  more  subacute  ox  chronic 
character.  In  some  of  these  cases  morbid  products  of  metabolism  may  be 
considered  to  be  the  cause  of  the  inflammation;  in  others  it  may  be  a  ques- 
tion of  necroses  and  softenings  resulting  from  the  anaemia  and  general 
cachexia,  which  so  frequently  accompany  these  diseases. 

A  peculiar  form  of  myelitis,  observed  in  workers  in  tunnels,  and  desig- 
nated Caisson  paralysis,  needs  special  mention.  As  a  result  of  a  sudden 
decrease  in  high  atmospheric  pressure,  there  is  a  development  of  gas  bubbles 
in  the  blood,  and  therewith  gas  embolism  occurs  in  the  blood-vessels  includ- 
ing the  vessels  of  the  spinal  cord.  Through  these  macroscopically  and 
microscopically  recognizable  embolizing  gas  bubbles  there  arise  numerous 
small  ischaemic  softening  foci.  Finally,  we  must  mention  the  rare  occur- 
rence of  softening  as  the  consequence  of  an  embolic  closing  of  the  aorta 
abdominalis,  also  embolic  and  thrombotic  occlusions  of  the  arteries  of  the 
spinal  cord. 

Symptomatology. — The  phenomena  of  the  disease  set  in  very  quickly 


4o4  DISEASES  OF  THE  SPINAL  CORD 

in  most  cases  of  acute  myelitis,  so  that  after  a  brief  prodromal  period,  during 
which  slight  pains  in  the  back  and  sacral  region,  weariness  and  paresthesias 
in  the  legs  are  complained  of,  the  symptoms  of  paralysis  develop  more  or 
less  suddenly  in  a  few  hours  or  in  the  course  of  several  days.  The  onset  is 
usually  with  fever;  fever  that  lasts  for  weeks  and  occasional  exacerbations 
of  fever  with  aggravation  of  the  spinal  symptoms  frequently  occur. 

Even  at  the  acme  of  the  disease,  the  inflammatory  focus  does  not,  as  a 
rule,  extend  symmetrically  over  the  entire  transverse  section,  but  leaves 
larger  or  smaller  areas  of  the  gray  and  the  white  matter  untouched.  Accord- 
ingly, the  grouping  of  symptoms  and  the  intensity  of  the  phenomena  of  the 
paralysis,  are  dependent,  not  only  on  the  height  of  the  localization,  but  also 
and  to  a  great  degree  on  the  manner  of  the  diffusion  to  the  individual  tracts 
of  the  spinal  cord.  In  discussing  the  spinal  phenomena,  we  shall  start  with 
the  most  frequent  form  of  myelitis  dorsalis. 

i.  The  disturbances  of  motility  stand  in  the  foreground  of  the  disease 
picture.  A  total  paraplegia  of  the  lower  extremities  develops  if  the  lateral 
pyramidal  and  anterior  columnar  tracts  are  destroyed  by  the  great  extent 
of  the  myelitic  focus.  In  an  incomplete  transverse  myelitis,  a  series  of 
muscles  will  still  be  able  to  carry  out  their  functions,  or  all  movements  of  the 
legs  will  still  be  possible,  but  with  decreased  strength.  The  paralysis  is  at 
first  flaccid;  but  very  soon  there  appears  a  muscular  stiffness,  a  spastic  condi- 
tion, which  can  lead  first  to  a  position  of  extension,  later  to  severe  flexion 
contractures  in  the  paralyzed  legs.  Not  only  in  the  beginning,  but  also  in 
the  further  course  of  the  disease,  motor  irritation  phenomena,  twitchings, 
appear  in  the  paralyzed  limbs,  which  partly,  at  least,  depend  on  the  increased 
reflex  excitability,  since  even  slight  irritations  of  the  skin,  such  as  moving 
the  bed  cover,  change  of  position,  etc.,  suffice  to  evoke  painful  muscular 
contractions;  partly,  also,  they  may  be  the  result  of  a  direct  irritation  of  motor 
fibres  of  the  spinal  cord.  .  The  participation  of  the  abdominal  muscles  is 
recognizable  even  in  its  slight  degrees  by  the  fact  that  the  patient  is  not 
able  to  rise  from  a  supine  position,  without  aiding  himself  with  his  hands. 

2.  The  tendon  reflexes  in  the  legs  are  usually  increased.  The  morbid 
increase  manifests  itself  in  that  when  the  knee-cap  or  the  tibia  of  the  extended 
leg  is  lightly  tapped,  a  contraction  of  the  quadriceps  muscle  results,  and  in 
that  patellar  and  ankle  clonus  may  be  excited.  A  permanent  loss  of  the 
tendon  phenomena  has  been  observed  only  in  those  cases  in  which  the 
disease  approximated  a  total  transverse  interruption  of  the  cord.  It  is 
noteworthy,  that  as  a  result  of  high  degrees  of  contractures,  the  excitation 
of  the  tendon  reflexes  may  be  made  difficult  or  impossible. 

The  cutaneous  reflexes  are  usually  retained  and  actively  increased,  so 
that  by  pinching  a  fold  of  the  skin  in  the  paralyzed  and  anaesthetic  regions 
muscular  contractions  of  that  or  even  of  the  opposite  extremity  result.  The 
plantar  reflex  shows  the  alteration  so  frequently  characteristic  of  a  spastic 


SYMPTOMATOLOGY  OF  MYELITIS  405 

paralysis,  dorsal  flexion  of  the  great  toe  when  the  sole  of  the  foot  is  stroked, 
especially  on  its  outer  side  (Babinski's  sign) .  In  more  extensive  transverse 
lesions  the  cutaneous  reflexes  like  the  tendon  reflexes  may  disappear  also. 
The  abdominal  reflexes  are  partly  or  entirely  lost,  if  the  reflex  arc  subserved 
by  the  posterior  roots  of  the  ninth  to  the  twelfth  intercostal  nerves  is  destroyed 
by  the  location  of  the  myelitic  focus  in  the  lower  dorsal  cord. 

3.  The  sensibility,  as  a  rule,  exhibits  considerable  disturbance,  even  to 
the  total  loss  of  all  qualities  of  sensation  in  the  legs  and  trunk,  up  to  a  level 
corresponding  to  the  region  subserved  by  the  segment  of  the  spinal  cord 
which  is  affected  by  the  focus.  If  the  process  is  restricted  to  a  smaller  part 
of  the  cross-section,  the  disturbance  in  sensation  extends  only  to  one  leg, 
or  parts  of  the  trunk,  or  consists  only  in  disturbances  of  certain  qualities  of 
sensation,  whereby  the  isolated  diminution  in  muscle,  tactile  and  pressure 
sense  points  to  a  predominant  disturbance  in  the  posterior  columns,  impair- 
ment of  the  temperature  and  pain  sensations,  to  an  affection  of  the  posterior 
horns. 

4.  Bladder  disturbances  are  frequently  an  early  symptom  of  acute  mye- 
litis, which  may  be  discovered  even  in  the  prodromal  stage.  A  participa- 
tion of  the  bladder  and  rectum  is  never  absent  in  any  of  the  more  extensive 
transverse  lesions.  In  the  beginning,  there  is  a  slight  difficulty  in  micturition, 
which  as  a  result  of  the  paralysis  of  the  detrusor  muscle  quickly  increases 
to  a  total  retentio  urines.  Overdistension  of  the  bladder  is  occasionally  not 
perceived  by  the  patients  at  all,  as,  because  of  the  lesion  of  the  sensory 
tracts,  the  conduction  of  the  irritation  impulses  arising  from  the  mucous 
membrane  of  the  bladder  and  going  to  the  cerebrum  no  longer  takes  place. 
As  further  consequence  of  the  excessive  filling  and  dilatation  of  the  bladder, 
there  appears  a  slow  dribbling  of  the  urine,  an  ischuria  paradoxa.  As  a 
rule,  after  a  certain  time,  the  mechanism  of  the  bladder  works  independ- 
ently, in  such  a  way  that  at  comparatively  regular  periods,  as  soon  as  the 
bladder  has  reached  a  certain  degree  of  fulness,  reflex  evacuation  takes  place. 
The  disturbance  in  rectal  activity  shows  itself  usually  as  a  high  degree  of 
sluggishness  of  the  bowels. 

5.  The  trophic  disturbances  of  the  skin  are  of  special  importance.  Far 
more  quickly  than  with  other  bed-ridden  patients  is  an  acute  appearance  of 
decubitus  observed  in  myelitis.  As  early  as  the  first  24  to  48  hours  after  the 
appearance  of  the  severe  phenomena  of  paralysis,  bed-sores  due  to  pressure 
may  develop,  so  that  careful  treatment  of  the  skin,  belongs  to  the  most  im- 
portant duties  of  the  physician,  from  the  very  beginning  of  the  disease. 
The  favorite  places  for  bed-sores  are,  beside  the  sacral  bones,  the  region 
of  the  trochanters,  the  heels  and  the  shoulder-blades,  and  the  inner  surfaces 
of  the  knees  where  they  rub  against  each  other.  Frequently,  also,  edema 
and  sclerodermoid  conditions  develop  during  the  longer  continuation  of  the 
disease. 


4o6  DISEASES  OF  THE  SPINAL  CORD 

6.  Of  cerebral  complications  of  a  focal  myelitis  only  neuritis  optica  is 
worth  mentioning.  Other  disturbances  in  the  realm  of  the  cerebral  nerves 
are  usually  absent  in  the  non-disseminated  form. 

If  the  seat  of  the  focus  is  not,  as  is  usual,  in  the  dorsal  portion  of  the  spinal 
cord,  but  in  the  cervical  or  lumbar  portion,  we  must  expect  certain  deviations 
from  the  disease  picture  just  described. 

Myelitis  cervicalis  is  the  rarest  and  most  dangerous  form  of  myelitis. 
If  the  focus  lies  above  the  cervical  enlargement  there  is  found,  besides  spastic 
paralysis  of  the  legs  and  the  usual  phenomena  of  a  dorsal  myelitis,  also  a 
spastic  paresis  of  the  arms;  a  bilateral  phrenicus  paralysis  is  followed  by 
death  through  suffocation.  If,  however,  the  focus  is  in  the  region  of  the 
cervical  enlargement,  there  develops — because  of  the  destruction  of  the  gang- 
lion cells  of  the  anterior  horn — &  flaccid,  atrophic  paralysis  of  the  arms  with 
electric  reaction  of  degeneration.  The  synchronous  appearance  of  oculo- 
pupillary  symptoms  due  to  sympathetic  paralysis — contraction  of  the  pupils 
and  of  the  palpebral  fissures — indicates  that  the  eighth  cervical  and  first 
dorsal  segments  are  also  affected. 

In  a  myelitis  lumbalis,  the  upper  extremities  are  quite  free;  there  exists 
&  flaccid  degenerative  paralysis  of  the  legs  with  weakening  or  absence  of  the 
tendon  reflexes.  The  paralysis  of  sensation  is  restricted  to  the  lower  extrem- 
ities alone ;  bladder  and  rectum  are  paralyzed. 

If  the  lower  sacral  cord  is  principally  affected,  it  is  preeminently  the  sciatic 
region  which  is  paralyzed — in  the  motor  aspect — and  the  sensory  disturb- 
ances extend  only  to  the  back  of  the  thighs  and  their  inner  half  to  the  lower 
third  on  the  buttocks  and  the  genital  organs.  Besides,  there  exist  disturb- 
ances in  bladder  and  rectum. 

Myelitis  disseminata  requires  special  discussion,  since  the  disease  picture 
may  exhibit  many  different  forms,  especially  when  greater  participation 
of  the  medulla  oblongata  and  the  brain  is  shown  to  exist  (Fig.  90).  When 
through  numerous  smaller  foci  a  larger  part  of  the  transverse  section  of  the 
spinal  cord  is  deprived  of  its  function,  the  disease  may  present  clinically  so 
completely  the  picture  of  an  acute  transverse  myelitis,  as  to  make  it  well- 
nigh  impossible  during  lifetime  for  a  diagnosis  of  disseminated  myelitis  to 
be  made.  Furthermore,  a  symptom  complex  may  develop  which  bears  the 
greatest  similarity  to  a  multiple  sclerosis  running  an  acute  course.  In  other 
cases,  an  acute  ataxia,  associated  with  loss  of  tendon  reflexes  and  bladder 
disturbances,  or  even  with  spastic  pareses,  occupy  the  foreground  of  the 
symptomatic  picture.  Occasionally,  also,  it  may  happen  that  in  the  begin- 
ning of  the  disease,  the  cerebral  phenomena  are  very  much  pronounced  as 
compared  with  the  symptoms  of  spinal  paralysis,  so  that  the  picture  of  a 
pure  encephalitis  may  be  believed  to  exist. 

Course. — As  a  rule,  myelitis  reaches  the  acme  of  its  development  within 
a  few  days  and  weeks;  death  may  occur  rapidly  or  possibly  an  improvement 


CHRONIC  MYELITIS  407 

may  ensue  (with  occasional  sharp  relapses,  which  are  accompanied  by 
renewed  fever),  which  may  lead  to  complete  recovery.  This,  however,  is 
rare  indeed;  usually  the  severe  phenomena  of  paralysis  are  but  partially 
recovered  from,  and  there  comes  to  be  a  permanent  condition  of  illness,  in 
the  course  of  which,  death  may  appear  at  any  time,  as  a  result  of  decubitus 
or  cystitis. 


Fig.  90. — Myelitis    disseminata.     Cross-section     through    the    pons  with  numerous  small  in- 
flammatory foci  grouped  around  the  vessels  (a).     (Author's  own  observation.) 

Chronic  Myelitis 

in  which  the  disease  picture  of  a  progressive  diffuse  transverse  myelitis 
develops  insidiously  from  the  first,  is  an  extremely  rare  disease.  Only  in 
isolated  cases,  heretofore,  could  be  demonstrated  an  anatomic  basis  for 
a  disease  of  the  spinal  cord,  exhibiting  the  picture  of  chronic  myelitis,  the 
presence  of  a  diffuse  chronic  myelitis.  In  the  great  majority  of  cases,  another 
disease  is  hidden  behind  the  symptom  complex  of  a  chronic  transverse 
myelitis,  a  multiple  sclerosis,  a  lues  spinalis,  a  combined  column  disease,  or  a 
compression  paralysis  of  the  spinal  cord. 

In  chronic  dorsal  myelitis,  the  initial  symptoms  are  first  of  all  a  slight 
tendency  to  fatigue  in  walking  with  increasing  stiffness  in  the  muscles  and 
abnormal  sensations,  paresthesias,  in  the  legs.  Actual  pains  are  only  tran- 
sitory, and  usually  present  only  in  slight  degree.  The  tendon  reflexes 
frequently  show  a  considerable  increase  with  or  without  accompanying  mus- 
cular rigidity.     The  objective  sensory  disturbances  usually  remain  slight, 


4o8  DISEASES  OF  THE  SPINAL  CORD 

as  compared  with  the  gradually  appearing  phenomena  of.  motor  paralysis. 
Bladder  and  rectal  disturbances  and  impotence  are  regular  concomitant 
phenomena  in  advanced  stages  of  the  disease.  The  duration,  as  a  rule,  ex- 
tends over  years;  there  may  be  a  standstill,  and  long  periods  of  improvement. 

Diagnosis. — For  the  diagnosis  of  acute  myelitis  transversa,  it  will  fre- 
quently be  of  importance  to  show  that  the  beginning  of  the  disease  was  con- 
nected with  certain  diseases,  infections,  etc.,  which,  as  we  know  from  experi- 
ence, play  an  aetiological  role  in  acute  myelitis.  Confusion  with  suddenly 
occurring  hemorrhage  of  the  spinal  cord  is  possible,  since  in  this  disease-, 
also,  prodromal  phenomena,  paresthesias,  twitchings  may  appear,  caused 
by  disturbances  in  circulation,  as  a  result  of  diseases  of  the  blood-vessels. 
Differentiation  from  a  pressure  paralysis  in  caries  or  vertebral  carcinoma, 
both  of  which  may  occasionally  develop  rapidly,  will,  as  a  rule,  cause  no 
difficulty,  since  the  neuralgic  prodromal  stage  and  eventually,  deformities 
of  the  spinal  column  afford  sufficient  points  for  diagnostic  differentiation. 
Schultze  observed  in  one  instance  that  a  tumor  of  the  spinal  meninges  (pos- 
sibly by  a  transitory  damming  back  of  the  cerebro-spinal  fluid)  might  rather 
suddenly  exhibit  the  phenomena  of  transverse  lesion,  and  thereby  simulate  an 
acute  myelitis.  In  such  cases,  only  further  observation,  the  proof  of  the 
steady  and  irresistible  progress  of  all  spinal  symptoms  will  make  diagnosis 
possible.  Separation  from  a  polyneuritis,  which  may  also  appear  in  connec- 
tion with  infectious  diseases,  will  be  called  into  question  only  when,  in  lumbar 
myelitis,  the  ganglion  cells  of  the  anterior  horns  are  destroyed,  and  atrophic 
degenerative  muscular  paralyses  are  present.  The  neuritic  pains  on  the 
one  hand,  and  on  the  other  the  bladder  disturbances,  which  are  very  rarely 
found  in  polyneuritis,  are  points  for  differentiation  between  them. 

Confusion  with  hysteric  paralyses  is  possible  especially  when,  associated 
with  the  usual  hysteric  phenomena,  there  is  present  a  real  transverse  myelitis. 
The  Babinski  sign  has  proved  to  be  a  sure  sign  for  distinguishing  between 
functional  and  organic  paralysis.  Beside  this,  the  change  in  the  severity 
of  the  phenomena  of  paralysis,  the  occasionally  striking  contrast  between 
the  ability  to  walk  and  the  mobility  of  the  legs  when  the  patient  is  lying  on 
his  back,  as  well  as  the  effect  of  suggestive  influences,  make  possible  the 
detection  of  the  hysteric  nature  of  the  disease. 

In  the  differential  diagnosis  of  chronic  myelitis,  the  most  important  dis- 
eases are  compression  paralyses  due  to  caries,  vertebral  tumors  and  tumors 
of  the  spinal  cord,  sclerosis  multiplex,  lues  cerebrospinalis  and  combined 
column  diseases.     We  would  refer  to  the  chapters  concerning  them. 

Prognosis. — This  is  always  serious  in  myelitis.  The  chances  of  pre- 
serving life  and  of  complete  or  partial  functional  recovery  are  the  less  favor- 
able, the  more  quickly  the  phenomena  of  a  total  transverse  lesion  are  devel- 
oped. Especially  dangerous  is  the  disease  of  the  upper  cervical  cord, 
because  of  the  possibility  of  the  occurrence  of  paralysis  of  the  diaphragm. 


TREATMENT  OF  MYELITIS  409 

Even  after  the  disappearance  of  the  acute  phenomena,  if  there  is  a  stationary 
condition  with  partial  functional  restoration,  the  disease  may  at  any  time 
terminate  fatally  in  consequence  of  decubitus  and  cystitis  with  their  resulting 
phenomena.  Those  cases  of  myelitis  which  appear  after  acute  infectious 
diseases  offer  the  best  chance  of  recovery.  Syphilitic  myelitis  which  develops 
soon  after  the  infection,  frequently  runs  quite  a  rapid  course. 

Treatment. — In  the  acute  stage  our  chief  aim  will  have  to  be  the  pre- 
vention of  decubitus  and  cystitis.  The  early  use  of  water  or  air  beds  and 
frequent  bathing  with  alcohol  and  water  of  those  parts  of  the  body  which 
are  especially  exposed  to  pressure,  act  as  prophylactics  with  respect  to 
pressure  bed-sores  which  so  frequently  develop  with  considerable  acute- 
ness  precisely  in  myelitis.  For  the  rest,  absolute  quiet  in  bed  at  the  begin- 
ning of  the  disease,  and  even  a  long  time  after  the  disappearance  of 
the  symptoms  of  the  spinal  paralysis,  is  necessary.  In  the  post-infectious 
forms,  diaphoretic  procedures  and  the  administration  of  preparations  of 
salicylates  are  recommended.  If  there  be  a  suspicion  of  syphilis,  iodide 
of  potassium  (gr.  x  t.i.d.)  should  be  tried;  mercurial  treatment  (3j  ung. 
hydrarg.),  in  which  there  is  always  a  possibility  that  the  Hg.  may  have  an 
injurious  effect  upon  the  more  severely  diseased  tissue,  must  be  stopped 
at  once  if  an  unfavorable  result  is  perceived.  In  previous  malarial  in- 
fection, quinine  and  arsenic  may  have  favorable  results.  After  lumbar 
punctures  we  have  observed  frequently  an  objective  improvement,  return 
of  the  reflexes,  increase  in  mobility. 

If  frequent  catheterization  is  necessary,  besides  very  painstaking  anti- 
sepsis, a  prophylactic  administration  of  urotropin  (gr.  v  t.i.d.)  is  advisable. 

In  the  stationary  stage  with  residuary  phenomena  of  pareses  and  slight 
spastic  conditions,  treatment  by  baths  is  to  be  tried  first.  There  is  need  of 
serious  warning  against  hot  baths.  Simple  warm  baths  {()2°-g6°  F.  and 
lasting  15  minutes)  are  most  useful,  which  in  case  of  more  severe  spastic 
phenomena  may  be  extended  over  a  long  time  (1-2  hours).  Treatment  at 
thermal  resorts  where  the  hot  springs  contain  carbonic  acid,  Oeynhausen, 
Nauheim,  and  others  like  Ragaz,  Teplitz,  Wildbad  are  to  be  recommended. 

As  a  means  of  comfort  for  the  patients  with  stationary  conditions  of 
paralysis,  the  galvanic  current  is  recommended  for  direct  treatment  of  the 
spinal  cord  (3  to  5  minutes  of  not  too  strong,  stabile  galvanic  current  on 
the  spinal  column)  and  for  muscular  nerve  stimulation  in  the  paralyzed 
extremities. 

We  may  expect  from  electricity  an  effect  that  will  further  recovery,  only 
when  the  phenomena  of  paralysis  are  themselves  on  the  point  of  retrogres- 
sion. If  a  bladder  disturbance  remains,  galvanic  treatment  may  be  tried, 
either  both  electrodes  over  the  lumbar  cord,  or  anode  on  the  lumbar  region, 
cathode  above  the  symphysis  or  upon  the  perineum.  The  strength  of  the 
current,  in  a  duration  of  5  to  10  minutes,  is  to  be  3  to  5  M.  A. 


4i o  DISEASES  OF  THE  SPINAL  CORD 

Supplement 

Spinal-cord  abscess,  an  extraordinarily  rare  disease,  develops  either 
after  an  injury  to  the  spinal  cord,  or  is  due  to  metastasis.  The  formation  of 
an  abscess  has  been  observed  after  putrid  bronchitis,  prostatic  suppuration, 
gonorrhoea,  malignant  endocarditis,  appendicitis.  Spinal-cord  abscess  is 
usually  of  central  location  and  may  become  very  extensive;  usually  it  is 
connected  with  purulent  meningitis,  which  occasionally  appears  only  later, 
so  that  lumbar  puncture  may  give,  at  first,  a  negative  result. 

The  disease  picture,  which  is  constructed  from  the  phenomena  of  a 
diffuse  transverse  disease  with  an  acute  onset,  affords  nothing  character- 
istic. Usually  it  will  be  concealed  behind  the  symptomatic  picture  of  an 
acute  myelitis  and  only  by  the  detection  of  other  purulent  foci,  synchronous 
general  pycemic  phenomena  and  a  purulent  exudate  found  by  lumbar  puncture, 
is  the  diagnosis  made  possible. 

(7).  DISEASES  OF  THE  CONUS   TERMINALIS  AND  THE  CAUDA 

EQUINA 

BY 

L.  R.  MULLER  (Augsburg) 

The  lowest  section  of  the  spinal  cord  and  the  nerve  roots  going  out  from 
it,  are  anatomically  so  different  from  the  other  parts  of  the  spinal  cord,  that 
the  diseases  there  have  a  peculiar  character. 

The  sacral  cord  with  the  conus  terminalis  is,  for  the  most  part,  situated 
behind  the  body  of  the  first  lumbar  vertebra  and  extends  downwards  only  to 
the  middle  of  the  second  lumbar  vertebra.  After  opening  of  the  spinal 
canal  and  of  the  dura  mater  the  lowest  division  of  the  spinal  cord  is  not, 
itself,  visible.  From  the  lumbar  enlargement,  spring  in  continuous  series 
thick  bundles  of  nerves,  which  all  running  downwards,  soon  become  so 
numerous  that  they  entirely  conceal  the  lowest  part  of  the  sacral  cord, 
the  conus.  A  separation  between  the  sacral  and  lumbar  cord,  such 
as  is  easily  to  be  seen  between  the  cervical  and  dorsal  portions,  or  the 
dorsal  and  lumbar  portions,  is  to  be  determined  only  with  chfficulty.  Only 
when  the  bundles  of  the  cauda  equina  are  traced  to  their  exit  from  the 
dural  sac  and  from  the  spinal  canal,  that  is  10  to  14  cm.  downwards,  can  the 
artificial  boundary  between  the  lumbar  and  sacral  cord  be  determined  by 
counting  the  separate  roots.  But  even  if  the  boundary  between  the  lumbar 
and  sacral  cord  can  not  be  recognized  morphologically,  theoretically,  at 
least,  a  distinction  must  be  maintained  between  the  nuclear  region  of  the 
plexus  lumbalis  and  that  of  the  plexus  sacralis  or  ischiadicus.  In  its  lower 
half,  the  sacral  cord  tapers  off  quickly;  that  part  of  the  spinal  cord  which 
embraces  the  third,  fourth  and  fifth  sacral  segment  and  both  coccygeal 


THE  CONUS  TERMINALIS  AND  THE  CAUDA  411 

segments,  is  called  conus  medullaris.  In  this  lowest  division  of  the  spinal 
cord,  the  relation  between  the  anterior  and  the  posterior  roots  is  not  so 
uniform  as  in  the  rest  of  the  spinal  cord.  If  more  than  double  the  number 
of  posterior  roots  enter  the  cord  here,  as  anterior  roots  spring  from  it,  this 
must  be  explained  by  the  fact  that  no  large  group  of  muscles  are  to  be 
innervated  from  the  third  sacral  segment  downward. 

Macroscopically,  then,  the  upper  boundary  of  the  conus  terminalis 
can  be  determined  only  arbitrarily,  or  not  at  all.  But  it  can  be  accurately 
determined  by  histological  examination.  In  the  third  sacral  segment,  the 
character  of  the  transverse  section  is  considerably  altered.  The  gray 
substance,  in  comparison  with  the  white,  is  excessively  developed.  As 
Fig.  91    shows,   the  plump,  broad    anterior  horns    are  abruptly  rounded 


*  5  * S>  *lmm 


;  \  -■ 


'vft-i'  '  W 


Fig.  91. — Cross-section  through  the  conus  terminalis. 

anteriorly,  and  no  longer  have  any  lateral  protuberance,  as  they  still  have 
in  the  upper  sacral  cord;  the  posterior  horns  fairly  bulge,  due  to  the  luxuriant 
development  of  the  gelatinous  substance.  The  ganglion  cells  in  the  anterior 
horns  are  very  scanty  here,  but  they  are  all  the  more  numerous  in  the  tran- 
sitional zone  between  anterior  and  posterior  horns;  from  here,  fibres  radiate 
directly  into  the  postero-lateral  columns,  and  into  the  roots  that  are  there 
situated.  The  lower  the  point  in  the  conus  from  which  the  sections  are 
taken,  the  larger  and  more  powerful  are  the  ganglion  cells  in  the  "inter- 
mediary zone."  In  the  arrangement  of  the  white  substance  also,  essential 
differences  may  be  established  in  the  conus  from  the  other  parts  of  the 
spinal  cord.  At  the  same  level  of  the  cord  at  which  the  great  ganglion  cells 
disappear  from  the  anterior  horn  and  new  groups  of  them  appear  in  the 
intermediary  zone,  one  finds  that  a  bundle  of  medullated  fibres  is  seen  to- 
pass  directly  from  the  posterior  columns  towards  the  front,  that  is,  ventrally 
towards  the  central  canal  and  on  both  sides,  increases  the  fibres  in  the 
intermediary  zone.  Here,  then,  and  this  characterizes  the  beginning  of 
the  conus,  no  posterior  gray  commissure  of  the  gray  substance  can  be  found. 


4i2  DISEASES  OF  THE  SPINAL  CORD 

Transverse  section  above  the  conus  shows  the  degeneration  in  the 
lateral  pyramidal  columns  does  not  extend  downwards  beyond  the  third 
sacral  segment.  On  the  other  hand,  in  the  posterior  columns  of  the  sacral 
cord  and  in  its  lowest  part,  in  the  conus  alongside  the  septum  medianum 
posticum  course  very  many  descending  conducting  paths,  whereas  in  the 
cervical,  dorsal,  and  lumbar  segments,  these  tracts  contain  almost  only 
centripetal  and  ascendingly  degenerating  fibres,  and  it  seems  to  be  these 
descending  fibres,  which  then  radiate  towards  the  front  into  the  gray  sub- 
stance, and  are  lost  in  the  intermediary  zone. 

In  the  conus  medullaris  the  centers  for  the  sexual  functions  and  for  the 
evacuation  of  bladder  and  rectum  were  always  supposed  to  have  been 
situated,  but  more  recent  investigations  cast  a  great  deal  of  doubt  upon 
the  correctness  of  this  hypothesis.  Thus,  it  can  be  demonstrated,  that  the 
act  of  parturition  may  proceed  normally,  even  after  disjunction  of  the  lowest 
section  of  the  spinal  cord.  Likewise  it  is  certain  that  after  the  lower  sacral 
cord  is  completely  destroyed,  erection  and  ejaculation  of  the  semen  into 
the  urethra  may  still  take  place;  as  a  result  of  the  paralysis  of  the  ischio- 
and  bulbo-cavernosi,  however,  the  semen  can  no  longer  be  ejaculated  from 
the  urethra,  but  dribbles  slowly  away. 

Neither  can  it  be  doubted  any  longer  that  the  last  nervous  centers 
subserving  the  bladder  and  the  rectum  are  situated  outside  of  the  spinal 
cord  in  the  sympathetic  nervous  system;  it  must  be  remembered  that  this 
theory  concerns  organs  with  involuntary  musculature  and  such  are  not 
innervated  directly  from  the  cerebro-spinal  system  anywhere  in  the  body. 
It  "is  true  that  the  reflex,  which  subserves  evacuation  of  urine  and  feces, 
can  be  carried  out  voluntarily  to  a  certain  degree.  But  the  formerly  gen- 
erally accepted  hypothesis,  that  one  center  for  the  detrusor  and  another  for 
the  sphincter  vesicae  are  situated  in  the  conus  medullaris,  is  probably  incor- 
rect. On  the  contrary,  we  must  conceive  of  the  automatic  process,  which 
leads  to  relaxation  of  the  sphincter  and  to  contraction  of  the  walls  of  the 
bladder,  as  being  entirely  excited  in  the  sympathetic  system.  We  cannot 
decide,  as  yet,  by  what  paths  this  reflex  is  evoked,  whether  by  way  of  the 
rami  communicantes  over  the  ganglion  mesenter  inferius  and  ganglion 
hypogastricum,  or  whether  possibly  this  process  might  be  aroused  by  a 
contraction  of  the  striped  musculature  situated  in  the  pelvis. 

The  conditions  underlying  the  evacuation  of  the  bowel-contents  are 
much  clearer.  By  abdominal  pressure,  the  column  of  feces  is  pushed 
downward  somewhat;  then  the  peristaltic  movement  of  the  ampulla  recti 
takes  hold  and  this  leads  to  the  ejection  of  the  excrement.  It  is  very 
improbable,  that  in  this  process,  a  center  for  defecation  in  the  conus  is 
actively  concerned.  True  it  is  that  the  transversely  striated  sphincter  ani 
externus  has  its  ganglion  cells  in  the  lower  division  of  the  spinal  cord,  just 
as  has  the  compressor  urethras,  which  is  also  under  the  influence  of  the  will- 


THE  CON  US  TERM  I  NAZIS  AND  CAUDA  413 

power;  spontaneous  peristaltic  movements  of  the  rectum  and  the  musculature 
of  the  bladder  may  therefore  be  repressed  to  a  certain  degree,  i.  e.,  it  is 
possible  to  retain  urine  and  feces,  even  when  desire  to  evacuate  exists. 
In  like  manner  the  centripetal  paths,  over  which  we  are  informed  as  to  the 
degree  of  fulness  in  the  bladder  and  the  ampulla  recti,  as  well  as  about  the 
processes  of  motility  taking  place  there,  probably  pass  through  the  conus 
terminalis.  Thus  is  explained,  that  when  the  lower  sacral  cord  is  diseased 
or  the  nerve  bundles  in  the  cauda  equina  passing  thither  are  interrupted, 
the  patient  is  not  aware  of  the  fulness  of  the  bladder  and  rectum,  and  is  not 
able  to  perform  the  automatic  process  necessary  for  the  ejection  of  the 
excrement  (retentio  urinae  et  fsecium).  Nor  is  he  able  to  inhibit  the  reflex, 
once  it  has  started,  as  in  diarrhoea  or  in  spontaneous  evacuation  of  urine 
by  contraction  of  the  voluntary  musculature  of  the  sphincters  of  the  anus 
and  the  bladder  (incontinentia  urinae  et  alvi).  The  disturbances  in  mictu- 
rition and  defecation  in  affections  of  the  conus  and  the  cauda  equina  do  not 
differ  from  those  which  develop  in  connection  with  transverse  affections 
in  the  rest  of  the  spinal  cord.  First  there  is  always  retention  of  urine  and 
feces,  later  there  appear  involuntary  automatic  evacuations. 

The  diseases  of  the  conus  medullaris  are  often  accompanied  by  those 
of  the  upper  sacral  and  the  lumbar  portion  of  the  cord,  or  they  attack  the 
bundles  of  roots  which  spring  from  these  parts  and  surround  the  conus; 
it  is  therefore  advisable  to  give  a  short  summary  of  the  situation  of  the  nuclear 
areas  of  the  individual  muscle  groups  in  the  lumbar  enlargement  of  the  spinal 
cord.  But  we  must  remark,  that  here  only  the  center  of  a  nuclear  realm  is 
indicated,  the  nuclear  realms  of  the  larger  muscle  groups  being  mostly 
extensive  and  reaching  over  several  segments. 

1.  Lumbar  segment:  lower  part  of  the  abdominal  muscles,  quadratus 
lumborum. 

2.  Lumbar  segment:  iliopsoas,  cremaster,  sartorius. 

3.  Lumbar  segment:  adductors  of  the  thigh. 

4.  Lumbar  segment:  quadriceps  femoris,  tibialis  anticus. 

5.  Lumbar  segment:  abductors  of  the  thigh  (glutaeus  medius  and 
minimus),  flexors  of  the  knee  (semimembranosus  and  semitendonosus 
and  biceps). 

1.  Sacral  segment :  glutaeus  maximus,  outward  rotator  of  the  hip,  extensors 
of  the  toes. 

2.  Sacral  segment:  gastrocnemius,  soleus,  peronei,  flexors  of  the  toes. 

3.  Sacral  segment:  ischio-  and  bulbocavernosus,  perineal  muscles 
(compressor  urethrae). 

4.  and  5.  Sacral  segment:  sphincter  ani  externus,  levator  ani. 
Figures    92-981    show   what    disturbances  in  sensibility    correspond    to 

1  Figs.  92-98  are  taken  from  a  previous  work  of  the  author's  (L.  R.  Mutter,  Investigations 
into  the  anatomy  and  pathology  of  the  lowest  segment  of  the  spinal  cord.  Deutsche  Zeitschrift 
fuer  Nervenheilkunde,  vol.  xiv). 


414 


DISEASES  OF  THE  SPINAL  CORD 


the  diseases  in  the  various  segments  of  the  lumbar  enlargement.  As  one 
may  see  from  Fig.  98,  the  nerves  from  a  small  oval  zone  about  the  anus 
reach  the  fourth  sacral  segment.  In  a  lesion  of  the  third  sacral  segment, 
the  anaesthesia  extends  also,  to  the  perineum,  the  dorsum  of  the  penis  and 
to  the  middle  parts  of  the  scrotum;  in  a  lesion  of  the  second  sacral  segment 
the  whole  external  genital  organs  are  anaesthetic,  the  insensitive  zone  in  the 
buttocks  has  become  considerably  larger,  extending  to  the  upper  portion  of 
the  posterior  aspect  of  the  thigh;  there  is  also  a  large  insensitive  area  on  the 
outer  portion  of  the  foot.  In  lesions  of  the  first  sacral  segment,  this  spreads 
to  the  outer  and  posterior  portion  of  the  leg  proper,  which  is  supplied  by  the 
peroneus  nerve.     Transverse  diseases  of  the  fifth  lumbar  segment  lead  to 


Fig.  9: 


-Lesion    at   the   level   of    the 
lumbar  segment. 


2d 


Fig.  93. — Lesion    at   the  level  of   the   3d   lumbar 
segment. 


the  typical  "riding  breeches"  anaesthesia  (Reithosenanaesthesie) .  In  all 
these  disturbances  of  sensibility,  the  testicles  in  the  anaesthetic  scrotum,  since 
they  are  innervated  by  the  spermatic  nerve  from  the  second  lumbar  segment, 
are  still  very  sensitive  to  pressure. 

Stroking  the  skin  in  the  immediate  vicinity  of  the  anus  causes  contraction 
of  the  sphincter  ani  and  the  levator  ani.  This  reflex,  the  anal  reflex,  has  its 
center  in  the  conus  terminalis,  hence  may  still  be  elicited  in  diseases  of  the 
upper  sacral  cord.  But  the  Achilles  tendon  reflex  is  lost  in  a  disturbance  at 
this  level  since  it  has  its  seat  in  the  region  of  the  epiconus,  in  the  second  sacral 
segment.  The  patellar  reflex  must  be  localized  much  higher  still,  in  the 
fourth  lumbar  segment. 

As  has  been  mentioned  before,  the  lower  end  of  the  conus  terminalis 
reaches  only  to  the  upper  margin,  or  at  most,  to  the  middle  of  the  second 
lumbar  vertebra;  from  that  point  on  the  fibre  bundle  of  the  cauda  equina, 


THE  CON  US  TERM  I  NAZIS  AND  CAUDA 


4*5 


which  is  about  as  thick  as  a  finger,  contains  only  the  filum  terminale.  The 
parallel  strands  of  the  cauda  equina  must  pass  for  a  long  way  inside  the 
spinal  canal,  in  order  to  reach  their  foramina  of  exit.  Thus  the  first  sacral 
nerve  arises  at  the  level  of  the  first  lumbar  vertebra  and  leaves  the  dural  sac 


Fig.  94. — Lesion  at  the  level  of  the  5th  lumbar         Fig.  95. — Lesion  in  the  1st  sacral  segment, 
segment. 


Fig.  96. — Lesion  in  the  2d  sacral  segment. 


Fig.  97. — Lesion  in  the  3d  sacral  segment. 


only  in  the  sacrum,  in  order  to  enter  the  pelvis  from  the  first  sacral  foramen. 
Therefore,  it  must  course  a  distance  of  14  cm.  within  the  dural  sac  of  an 
adult.  A  cross-section  through  the  cauda  equina,  at  the  level  of  the  conus 
terminalis  strikes  besides  all  the  sacral  nerves,  all  the  lumbar  roots  also  with 
the    exception    of    the    first.     Naturally,  the  lumbar  roots  which  escape 


4i6 


DISEASES  OF  THE  SPINAL  CORD 


higher  up,  are  situated  at  the  outer  side  in  the  bundle  of  the  cauda  equina, 
while  the  sacral  fibres  which  escape  farther  down,  are  medially  situated. 

The  sensory  roots  form  their  spinal  ganglion  only  after  their  escape  from 
the  dural  sac;  it  reaches  a  quite  considerable  size  in  the  lumbar  nerves,  but 
especially  so  in  the  sacral  nerves.  Now,  finally,  after  the  sensory  root  has 
passed  through  the  spinal  ganglion,  it  unites  with  the  anterior  roots  to  form 
the  peripheral  nerve.  The  fibres  of  the  cauda  equina,  therefore,  are  not,  as 
often  happens,  to  be  regarded  as  peripheral  nerves,  but  as  root  fibres,  i.  e., 
in  the  cauda,  the  motor  and  sensory  bundles  course  separated  from  each 
other;  we  may  easily  convince  ourselves  in  processes  in  which  only  the 
sensory  tracts  degenerate  (for  instance  in  tabes)  that  these  sensory  bundles 
are  situated  all  together,  in  one  posterior  field,  while  the  motor  roots  are 


VW1 

Fig.  98. — Lesion  in  the  4th  sacral  segment. 


situated  farther  front,  also  joined  in  groups.  It  is  clear  that  this  fact  is  of 
great  importance  for  the  diagnosis  and  localization  of  the  disturbances  with 
which  we  are  here  concerned  (caudal  diseases). 

After  the  escape  of  the  caudal  fibres  from  the  spinal  canal  one  distinguishes 
five  pairs  of  lumbar  nerves,  the  first  of  which  pass  out  between  the  first  and 
second  lumbar  vertebrae,  the  last  between  the  fifth  lumbar  vertebra  and  the 
sacrum;  five  pairs  of  sacral  nerves  which  radiate  outwards  through  the  foramina 
sacralia  anteriora  and  posteriora  and  finally  one  coccygeal  nerve,  which  after 
passing  the  hiatus  sacralis  courses  downwards.  Now,  while  the  intercostal 
nerves  form  no  plexus,  the  outgoing  lumbar  and  sacral  nerves  are  mingled, 
each  in  a  plexus,  and  now  for  the  first  time  they  separate  into  the  real  periph- 
eral nerves.  Thus,  the  distribution  of  fibres  in  the  cauda  equina  and  the 
order  of  their  origin  from  the  spinal  cord  is  different  from  the  arrangement  in 
thejDeripheral  nervous  system.     The  plexus  lumbalis  is  formed  by  the  four 


ETIOLOGY  OF  DISEASES  OF  THE  CONUS  TERMINALIS      417 

upper  lumbar  nerves,  the  sacral  plexus,  the  large  plexus  situated  on  the 
anterior  surface  of  the  pyriformis  muscle,  is  made  up  from  parts  of  the  fourth, 
from  the  fifth  lumbar  nerve  and  from  the  upper  four  sacral  nerves.  The 
last  sacral  nerve  and  the  coccygeal  nerve  unite  to  form  the  plexus  coccygeus. 
The  nervus  ano-coccygeus  thus  formed  supplies  the  skin  in  the  neighborhood 
of  the  tip  of  the  coccyx. 

To  summarize  from  the  innervation  of  the  pelvis,  what  is  important  for 
diagnosis,  we  must  point  out  that  the  anterior  and  external  portions  of  the 
pelvis  and  thigh  get  all  their  sensory  supply  from  the  lumbar  plexus;  only  the 
penis  and  the  scrotum  are  supplied  by  the  pudendal  nerve,  which  springs 
from  the  sacral  plexus.  The  posterior  portion  of  the  pelvis,  also,  or,  better, 
of  the  buttocks,  is  innervated  by  the  ramus  iliacus  of  the  nervus  ilio-hypo- 
gastricus  and  by  the  nervus  cutaneus  femoris  lateralis  from  the  lumbar  plexus; 
on  the  other  hand,  an  oval  zone,  about  the  size  of  a  saucer,  lying  around  the 
anus,  and  the  perineum  gets  its  sensory  supply  from  the  sacral  plexus  and 
the  coccygeal  plexus. 

The  conditions  within  the  pelvis  are  still  more  complicated.  Of  the 
muscles  there  situated,  the  psoas,  the  iliacus  internus,  the  obturator  externus, 
the  cremasier  and  the  tunica  dartos  are  innervated  from  the  lumbar  plexus, 
all  the  other  small  pelvic  muscles  (M.  pyriformis,  M.  obturator  internus, 
Mm.  gemelli),  the  musculature  of  the  perineum,  the  anus,  and  the  large 
muscles  of  the  buttocks  receive  their  nerves  from  the  sacral  plexus. 

The  diseases  of  the  conus  terminalis  are  brought  about  by  various  causes. 
Since  the  conus  terminalis  is  part  of  the  substance  of  the  spinal  cord,  all 
pathologic-anatomical  processes,  which  attack  the  rest  of  the  medulla  spinalis, 
may  be  localized  occasionally  in  this,  its  lowest  part,  as  well.  As  a  matter  of 
fact,  isolated  cases  of  myelitis,  haematomyelia,  multiple  sclerosis,  and  various 
sorts  of  tumors  have  been  described  in  the  conus  medullaris.  In  the  great 
majority  of  cases,  however,  traumatisms  are  responsible  for  the  affections  of 
the  conus.  The  first  lumbar  vertebra,  behind  which  the  conus  is  situated — 
as  a  result  of  the  static  relations  of  the  spinal  column,  in  its  capacity  as  a 
transitional  vertebra  from  the  rigid,  backwardly  curved  dorsal  spinal  column 
to  the  movable  lordotic  lumbar  spinal  column — suffers  with  especial  fre- 
quency compression  fracture  from  violent  jolts,  i.  e.,  falls  upon  the  legs  or  the 
buttocks.  By  such  an  injury,  the  spinal  canal  is  narrowed  and  the  lowest 
section  of  the  spinal  cord  crushed.  The  fibres  of  the  cauda  equina,  which 
surround  the  conus,  being  far  tougher  and  more  capable  of  resistance,  usually 
do  not  surfer,  when  the  first  lumbar  vertebra  is  broken,  since  they  are  able 
to  get  out  of  the  way  in  time,  so  that  pure  cord  lesions  without  participation  of 
the  roots  have  been  observed.  Fig.  99  shows  the  cross-section  of  a  dural 
sac,  broadly  flattened  out  by  pressure  from  a  compression  fracture  of  the  first 
lumbar  vertebra;  the  cauda  bundles  are  very  well  preserved,  the  destroyed 
conus  is  replaced  by  gliar  and  fibrous  scar  tissue.  The  preparation  is  taken 
27 


4i8  DISEASES  OF  THE  SPINAL  CORD. 

from  a  patient,  who  by  falling  from  a  height  of  10  m.  sustained  a  fracture  of 
his  first  lumbar  vertebra. 

Occasionally,  after  a  concussion,  the  typical  symptoms  of  a  conus  affection 
appear,  though  it  is  impossible  to  discover  the  presence  of  a  deformity  in  the 
spinal  column.  A  possible  explanation  for  such  cases  might  lie  in  the  fact 
that  there  had  been  no  fracture,  but  merely  a  momentary  elastic  displacement 
of  the  upper  lumbar  spinal  column,  which,  however,  sufficed  to  crush  the 
conus.  On  the  other  hand,  the  following  hypothesis  has  been  advanced  by 
Fischler:  The  fall  is  followed  by  excessive  forward  flexion  of  the  trunk,  which 
in  turn,  causes  violent  bending  in  the  spinal  cord,  so  that,  at  the  place  where 
the  caudal  roots  enter  the  conus,  there  may  be  lacerations  and  secondary 
hemorrhages  into  the  cord. 


Cicatricial  tissue. 


Fibre  of  the  cauda 
equina. 


Fibre  of  the 
cauda  equina. 

Dura  mater. 

Glial  substance  of  the  4th  sacral. 
Fig.  99. — -Cross-section  through  the  dural  sac  after  crushing  of  the  1st  lumbar  vertebra. 

Besides  traumatism,  the  other  causes  of  disease  play  but  a  slight  part. 
Now  and  again  the  symptoms  of  an  affection  of  the  conus  develop  spontane- 
ously, and  with  comparative  rapidity,  so  that  the  existence  of  a  myelitis 
must  be  suspected.  Only  rarely  do  tumors  develop  in  the  conus  itself;  on 
the  other  hand,  it  occurs  more  frequently  that  the  conus  is  compressed  by 
tumors  starting  in  the  cauda  equina  or  the  vertebrae,  by  pachymeningitis,  or . 
by  tuberculous  processes.  The  literature  does  not  as  yet  furnish  cases  of 
syringomyelia,  poliomyelitis,  sclerosis  multiplex  of  the  conus  medullaris, 
which  were    positively  identified  as  such  by  postmortem    examinations. 

The  clinical  phenomena  of  conus  diseases  are  limited  to  an  anaesthesia 
over  the  sacrum,  anus,  perineum,  in  the  genitalia,  and  to  a  disturbance  in 
the  functions  Of  the  rectum,  the  bladder  and  the  sexual  organs;  nearly  always 
there  appears,  as  has  been  already  stated,  retention  of  urine  and  feces. 
After  ischuria  paradoxa  has  persisted  for  some  weeks,  or  has  been  combated 
by  catheterization,  frequent  spontaneous  evacuations  of  urine  occur.  At  a 
certain  degree  of  fulness  in  the  bladder,  automatic  evacuation  is  induced, 
Shortly  before,  the  patients  usually  have  a  certain  indefinite  sensation  in  the 


SYMPTOMATOLOGY  OF  DISEASE  OF  THE  CONUS  419 

abdominal  region,  so  that,  if  a  receptacle  is  at  hand,  they  may  avoid  getting 
soiled  by  the  urine.  But  voluntary  excitation  or  suppression  of  micturition 
is  and  remains  impossible.  Closure  of  the  sphincter  is,  however,  not  so 
secure  as  in  the  healthy,  so  that  during  coughing,  sneezing,  or  more  violent 
movements,  urine  generally  escapes.  Retention  of  feces,  on  the  other  hand, 
remains  permanently;  usually,  there  is  evacuation  only  after  the  administra- 
tion of  a  purgative.  After  taking  the  purgatives,  the  patients  must  remain 
for  hours  on  the  toilet,  because  they  have  no  sensation  of  the  oncoming 
defecation,  and  they  can  not  hold  it  back  even  for  a  moment  because  of  the 
paralysis  of  the  external  sphincter  ani.  Since  the  patients  are  not  able  to 
retain  rectal  injections,  the  constipation  can  not  be  combated  by  enemata. 
The  paralysis  of  the  sphincter  ani  externus  is  also  evinced  by  the  fact  that 
the  anal  reflex  is  always  lost  in  affections  of  the  conus.  The  sexual  functions, 
immediately  after  an  injury  to  the  conus,  and  as  long  as  severe  general 
phenomena  like  ischuria  or  acute  cystitis  persist,  are  entirely  absent. 
Later  on,  in  spite  of  the  disjunction  of  the  lower  section  of  the  spinal  cord, 
penile  erection  may  occur  and  evacuation  of  the  semen  into  the  urethra, 
though,  as  a  rule,  the  sensory  impressions  and  orgasm  are  absent.  From  the 
urethra,  the  semen  dribbles  but  slowly;  hence  it  is  always  possible  that  such 
patients  may  have  fructifying  cohabitation. 

In  pure  affections  of  the  conus,  i.  e.,  if  the  pathologic-anatomical  process 
is  restricted  to  the  lowest  three  sacral  and  the  coccygeal  segment,  there  are 
no  disturbances  in  sensation  or  phenomena  of  paralysis  in  the  lower  extrem- 
ities. Locomotion  therefore  is  in  no  way  interfered  with.  Very  frequently, 
however,  the  upper  sacral  cord,  or  even  the  lowest  lumbar  cord,  are  simul- 
taneously affected,  and  then  there  are  added  to  the  bladder  and  rectal  dis- 
turbances, paralyses  of  the  peronei  and  flattening  and  functional  loss  of  the 
glutaeus  maximus  and  of  the  flexors  of  the  knee;  the  loss  of  sensation  about 
the  anus,  of  the  perineum  and  the  genitalia,  are  supplemented  by  the  anaes- 
thetic zones,  represented  in  Figs.  92-98. 

After  traumatic  lesions  of  the  conus,  the  morbid  changes  in  the  cord, 
such  as  hemorrhage,  serous  infiltration,  edema,  or  reactive  inflammation 
extend  nearly  always  to  the  middle  of  the  lumbar  enlargement.  In  time, 
however,  the  reparable  disturbances,  and  together  with  them,  the  phenomena 
of  paralysis  and  the  loss  of  sensation  disappear  in  the  lower  extremities,  and 
only  those  symptoms  remain  which  were  excited  by  crushing  the  substance 
of  the  cord. 

Minor  denominates  as  the  epiconus  that  part  of  the  lumbar  enlargement 
which  is  situated  immediately  above  the  conus  and  corresponds  to  the 
5th  lumbar  and  the  1st  and  2nd  sacral  segments.  Disease  pictures  are 
described  by  this  author,  in  which  the  paralysis  is  confined  to  the  realms  of 
the  peronei  and  glutaei,  so  that  even  the  knee  reflexes,  on  the  one  hand,  and 
the  control  over  the  sphincters  of  the  bladder  and  rectum,  on  the  other,  are 


42o  DISEASES  OF  THE  SPINAL  CORD 

retained.  Such  a  symptom-complex  is  nearly  always  caused  by  acute 
poliomyelitis. 

In  transverse  diseases  in  the  lumbar  cord,  caused,  as  they  may  be,  by 
inflammatory  or  traumatic  myelitis,  or  by  tumorous  formations,  there  results, 
from  the  interruption  in  conduction,  also  paralysis  of  the  muscular  groups 
innervated  by  the  sacral  plexus.  But  here  the  type  of  paralysis  will  be  spastic 
(ankle  clonus,  retention  of  the  Achilles  reflex)  whereas  in  the  realm  of  the 
lumbar  plexus,  degenerative,  i.  e.,  atrophic  paralyses  may  be  demonstrated 
(absence  of  the  patellar  tendon  reflex) . 

If  the  motor  disturbances  predominate  in  the  diseases  of  the  conus,  the 
sensory  irritation  phenomena  are  characteristic  of  the  caudal  diseases. 
Scarcely  any  other  disease  is  associated  with  such  painful  conditions,  as  the 
gradual  compression  of  the  bundle  of  the  cauda  equina,  as  it  results  from 
tumors.  The  pains  are  referred  to  the  anus,  the  perineum,  and  the 
genitalia,  and  frequently  radiate  to  the  legs  proper  and  the  peroneal  realm. 
In  advanced  cases,  the  corresponding  cutaneous  areas  are  insensitive,  as  a 
result  of  the  break  in  conduction  in  the  roots  of  the  cauda,  but  nevertheless 
there  are  still  pains  in  this  region,  and  the  condition  is  then  spoken  of  as 
"anaesthesia  dolorosa." 

The  disturbances  caused  by  caudal  diseases  appear  gradually,  especially 
when  they  are  caused  by  tumors.  At  first,  there  are  pains  only  with  certain 
movements  of  the  body,  or  by  pressure  on  a  vertebra;  later  they  appear 
spontaneously,  become  almost  continuous,  and  are  increased  to  unbearable 
violence. 

Although  the  cauda  equina  contains,  in  its  upper  part,  roots  which  come 
from  the  lumbar  cord,  nevertheless,  the  pains  in  compression  of  the  cauda 
equina  are  nearly  always  restricted  to  the  realm  of  the  sacral  roots.  This 
fact,  taught  us  by  experience,  is  not  easily  explained,  since  it  is  only  natural 
to  suppose,  that  in  a  space-contracting  process  in  the  canal  of  the  lumbar 
spinal  column  the  roots,  situated  at  the  outer  side  would  be  affected 
likewise. 

In  the  cauda  equina,  as  was  stated  above,  the  sensory  and  motor  tracts  are 
still  separated.  Thus  it  is  comprehensible,  that  sensory  irritation  phenom- 
ena may  exist  without  motor  disturbances.  Pareses  and  paralyses  develop 
much  later,  if  at  all.  The  paralyses  are  flaccid  and  usually  not  strictly 
symmetrical,  i.  e.,  more  pronounced  on  one  side  than  on  the  other.  The 
disturbances  in  bladder  and  rectum  caused  by  caudal  affections  are  in  no 
wise  different  from  those  which  appear  in  connection  with  conus  diseases, 
except,  possibly,  that  their  onset  is  more  gradual;  before  ischuria  supervenes 
the  evacuation  of  the  urine  is  made  very  difficult  for  a  long  time. 

As  cause  of  their  origin,  traumatism  in  lesions  of  the  cauda  equina  plays 
not  nearly  so  important  a  part  as  in  those  of  the  conus.  In  the  first  place, 
the  lower  lumbar  vertebras  and  the  os  sacrum,  are  not  so  frequently  exposed  to 


DIAGNOSIS  OF  DISEASE  OF  THE  CON  US  AND  CAUDA       421 

fractures  as  the  first  lumbar  vertebra,  which  is  especially  endangered  in  falls 
on  the  legs,  or  on  the  buttocks;  then  the  tougher  nerve  roots,  which  are  more 
capable  of  resistance,  evade  injury  in  compression  of  the  spinal  cord  more 
easily  than  can  the  tender  substance  of  the  sacral  cord.  Very  often,  however, 
tumors  cause  injuries  of  the  caudal  roots.  All  sorts  of  growths,  like  neuroma, 
lymphangioma,  fibromyxoma,  enchondroma,  exostoses,  cysticerci,  etc.,  may 
develop  within  the  lower  spinal  canal,  inside  and  outside  of  the  dural  sac. 
But  especially  frequently  metastases  lead  to  compression  of  the  caudal  fibres 
and  above  all  carcinomata  and  sarcomata  show  an  inclination  to  second- 
ary tumor  formation  in  the  lumbar  spinal  column,  and  in  the  os  sacrum. 
On  the  other  hand,  tubercular  and  gummatous  processes  occur  far  more 
rarely  in  the  lower  part  of  the  vertebral  column  than  in  the  dorsal  or  cervical 
parts. 

It  is  to  be  remembered  that  tumors  outside  of  the  spinal  canal  may  also  lead 
to  injury  of  the  nerve  tracts  here  concerned.  A  tumor  located  in  the  pelvis, 
and  attached  to  the  sacrum  will,  as  it  grows,  attack  the  fibres  which  pass  to 
the  bladder,  the  genitalia,  and  the  rectum.  Such  disturbances  assume  the 
character  of  plexus  paralyses.  For  the  nerve  tracts  are  not  usually  affected 
by  the  injury,  at  a  place  where  the  bundles  are  already  leaving  the  plexus  to 
separate  into  single  nerves,  but  are  injured  immediately  after  they  leave  the 
spinal  column,  that  is,  in  the  plexus,  or  before  they  have  joined  to  form  it. 

Finally  it  must  be  mentioned  that  diseases  of  the  peripheral  nerves  present 
similar  clinical  phenomena  as  affections  of  the  plexus  or  cauda.  Frequently, 
cases  of  sciatica,  or  of  coccygodynia  can  scarcely  be  distinguished  from  the 
initial  difficulties  caused  by  compression  of  the  fibres  of  the  cauda  equina, 
or  of  the  sacral  and  coccygeal  plexuses. 

From  the  facts  here  presented,  one  may  appreciate  what  difficulties 
differential  diagnosis  may  present,  in  cases  in  which  one  must  decide 
whether  the  lower  portion  of  the  spinal  cord  itself,  or  its  surrounding  and 
outgoing  roots,  in  their  long  course,  or,  finally  the  plexus  and  its  peripheral 
processes  are  injured.  But  differential  diagnosis  has  not  only  theoretical 
interest,  but  is  of  great  practical  importance,  also.  Thus,  the  prognosis 
changes  considerably,  as  the  location  of  the  seat  of  the  disease  varies.  In  a 
sciatica,  the  prognosis  is  usually  favorable;  if  the  same  subjective  disturbances 
are  caused  by  the  formation  of  a  tumor  in  the  lower  spinal  canal,  one  must 
count  upon  an  aggravation  of  the  disease.  Diseases  of  the  lower  spinal  cord 
itself  usually  run  their  course  rapidly,  but  their  resulting  phenomena  remain 
stationary,  after  the  reactive  phenomena  have  disappeared,  i.  e.,  improvement 
and  cure  are  excluded,  but  neither  is  an  aggravation  of  the  disease  to  be 
feared,  unless  complications  appear  through  decubitus,  or  through  cystitis 
and  pyelitis.  In  our  discussion  of  differential  diagnosis,  we  must  emphasize 
again  the  fact,  that  in  diseases  of  the  cord  itself,  the  motor  phenomena  of 
functional  loss  predominate,  that  these  are  usually  symmetrical  and  exhibit  a 


422  DISEASES  OF  THE  SPINAL  CORD 

segmentary  arrangement.  The  sensory  disturbances,  in  diseases  of  the 
conus,  are  restricted  to  loss  of  sensation;  the  anesthesia  is  frequently  dissoci- 
ated, i.  e.,  includes  only  certain  qualities  of  sensation,  such  as  pain  and 
temperature.  The  Brown-Sequard  symptom  complex  points  definitely  to  a 
localization  of  the  disease  in  the  lumbar  enlargement.  Furthermore, 
the  sensory  irritation  symptoms  are  lacking  in  conus  diseases.  These, 
however,  predominate  in  the  clinical  picture,  when  a  caudal  affection  is  con- 
cerned. If  the  pains  are  permanently  unilateral,  one  must  consider  whether 
a  plexus  disease,  or  a  neuralgia  of  a  peripheral  nerve,  f.  i.,  ischialgia,  is  not 
present.  Sensitiveness  of  the  musculature  to  pressure  speaks  for  neuritis; 
in  the  latter  case,  the  paralyses  are  usually  definitely  located  in  the  area  of 
one  or  more  peripheral  nerves.  The  disturbances  in  the  bladder  and  rectum 
can  not  be  used  in  a  differential  diagnosis  between  "conus"  and  "cauda." 
Only  this  one  fact  points  to  a  caudal  affection,  if  together  with  ischuria, 
and  retentio  faecium,  violent  pains  radiate  to  the  perineum.  In  diseases  of 
the  peripheral  nerves,  as  in  coccygodynia,  in  sciatica,  or  polyneuritis,  dis- 
turbances in  evacuation  of  urine  and  feces  are  always  lacking. 

In  typical  cases,  the  differential  diagnosis  between  diseases  of  the  cauda 
and  conus  presents  no  difficulties  too  great  to  be  overcome.  But  if  a  caudal 
lesion  had  been  diagnosed,  then  the  difficult  decision  must  be  made,  at 
what  height  of  the  cauda  the  lesion  is  to  be  sought.  The  rule  may  be  estab- 
lished here,  as  in  diseases  of  the  spinal  cord,  to  look  for  the  disturbance 
at  as  high  a  point  as  possible.  The  nerves,  which  subserve  the  bladder  and 
rectum,  may  be  injured  throughout  their  entire  course  through  the  cauda, 
and  it  has  been  shown  several  times,  that  in  diseases  of  the  cauda  located 
high,  these  nerves  situated  in  the  middle  and  passing  out  farthest  down, 
usually  suffer  -first.  .  The  lesion  of  the  cauda  must  at  least  be  localized  at  a 
height,  which  corresponds  to  the  exit  of  the  uppermost  roots,  in  which 
irritation  phenomena  are  still  demonstrable. 

If  a  disease  of  the  cauda  equina  and  the  conus  medullaris  are  synchro- 
nously present,  a  more  exact  diagnosis  will  usually  be  impossible. 

Treatment  is  absolutely  impotent  when  facing  myelitic  processes  in  the 
lower  division  of  the  spinal  cord,  whether  they  be  of  inflammatory  or  trau- 
matic character,  whether  they  be  recent  or  partly  healed.  Likewise,  when 
a  tumor  has  caused  pressure  atrophy  of  the  cord,  no  result  is  attained  by 
its  removal. 

Conditions  in  the  cauda  equina  are  different.  There,  by  extirpation 
of  the  compressing  tumor,  an  amelioration  of  troubles,  indeed,  under 
certain  conditions,  complete  recovery  may  be  effected.  The  motor  roots 
of  the  cauda  have  their  trophic  centre  in  the  spinal  cord,  and  the  sensory, 
in  the  spinal  ganglia,  and  thence,  a  restoration  of  fibres,  and  at  the  same 
time,  of  the  functions  may  result.  The  operative  technique  also  is  not  excep- 
tionally difficult.     But  permanent  results  are  opposed  by  the  fact  discussed 


TREATMENT  OF  DISEASES  OF  THE  CON  US  AND  CAUDA   423 

above,  that  in  the  lower  spinal  canal,  we  rarely  have  to  deal  with  isolated 
tumors;  on  the  contrary,  it  is  usually  a  matter  of  metastases.  Operation  is 
absolutely  hopeless,  if  tumor  masses  penetrate  the  vertebral  bones.  Natu- 
rally, with  the  first  symptoms  of  a  caudal  compression,  treatment  with 
iodide  of  potassium  and  mercury  will  always  be  instituted  at  once,  assuming 
the  possibility  of  a  gummatous  change  or  a  pachymeningitis  specifica  of  the 
lower  dural  sac  being  present. 


IV 
THE  MYOPATHIES 

WITHOUT  DEMONSTRABLE  CHANGE  IN  THE  NERVOUS  SYSTEM 

BY 

HANS  CURSCHMANN  (Mainz) 

Under  the  term  myopathies,  we  can  group  together  a  series  of  disease 
pictures,  all  of  which  are  of  rather  rare  occurrence :  dystrophia  musculorum 
progressiva  (Erb),  myotonia  congenita  (Thomsen),  myasthenia  pseudo- 
paralytica  and  lastly  a  disease,  that  has  been  known  only  for  a  few  years — ■ 
congenital  myatonia  (Oppenheim).  All  four  are  severe  afflictions  of  the 
function  of  movement;  some  forms  are  accompanied  by  trophic  disturbances 
of  the  musculature  (dystrophy,  amyotrophic  myotonia),  others  run  their 
course  without  any  macroscopic  changes  in  it  (myasthenia,  myotonia  con- 
genita, myatonia  (Oppenheim).  Their  disturbances  in  movement  vary  con- 
siderably ;  in  part  they  are  directly  antagonistic  (myotonia  and  myasthenia) . 
In  one  respect,  however,  all  four  diseases  are  alike:  in  the  macroscopic 
and  microscopic  intactness  of  the  nervous  system,  on  the  one  hand,  and  in  the 
well-characterized  macroscopic,  or,  as  is  mostly  the  case,  only  microscopic 
changes  in  the  musculature.  It  has  been  attempted,  also,  to  include  the  first 
three  diseases  under  one  pathogenetic  hypothesis  (Lundborg)  without,  how- 
ever, being  able  to  prove  their  common  parathyreogenic  origin. 

i.  DYSTROPHIA  MUSCULORUM  PROGRESSIVA  (ERB) 

From  the  confusion  of  the  various  forms  of  muscular  wasting  that  still 
reigned  in  the  time  of  Friedreich,  Duchenne,  Charcot  and  others,  ErVs  search- 
ing hand  has  extricated,  under  the  above  name,  a  definite  group  of  pure 
myopathic  atrophies  and  pseudo-hypertrophies.  Even  though  the  usual 
division  into  various  sub-forms  (infantile,  pseudo-hypertrophic  form, 
juvenile  form,  etc.),  is  to  be  retained  in  these  diseases,  the  fact  must  never- 
theless be  emphasized,  that  transitional  forms  occur  quite  as  frequently,  as 
the  pure  and  separate  forms,  and  this  always  brings  the  unity  of  Erb's 
disease  picture  to  clear  recognition.  One  may  therefore  well  dispense  with 
distinctions  between  still  more  separate  forms  (Leyden-Moebius,  Duchenne- 
Dejerine,  etc.). 

Definition. — Erb's  dystrophy  embraces  forms  with  the  following  peculi- 
arities:    i.  Beginning  of  the  disease  in  childhood  and  youth  (from  earliest 

424 


PROGRESSIVE  MUSCULAR  DYSTROPHY 


425 


infancy  to  the  end  of  the  second  decade,  very  rarely  in  the  thirties  and  forties) ; 
2.  very  frequent  (but  by  no  means  constant)  familial  appearance  in  such  a 
manner  that  the  disease  is  usually  found  in  several  brothers  and  sisters,  but 
there  is  but  rarely  transmission  from  ancestors  to  descendants;  3.  atrophies 
and  pseudo-hypertrophies  regularly  attack  definite  groups  of  muscles,  the 
atrophies  usually  definite  muscles  of  the  chest,  shoulder-girdle,  back,  arms, 
buttocks  and  thighs,  often,  too, 
those  of  the  face,  while  the  distal 
parts  of  the  limbs  are  nearly  always 
spared  to  the  elbow  and  to  the 
knee  (in  striking  contrast  to  spinal 
muscular  atrophy) ;  the  pseudo-hy- 
pertrophy prefers  definite  muscles 
of  the  arm  and  above  all,  the  mus- 
cles of  the  calf  of  the  leg;  4.  the 
clinical  signs  of  degenerative  atrophy 
(fibrillary  tremors,  electrical  and 
mechanical  reactions  of  degenera- 
tion) are  nearly  always  absent;  5. 
the  following  other  symptoms  of 
functional  loss  of  the  central  and  per- 
ipheral neuron  are  always  absent, 
also:  participation  of  the  sensory 
cerebral  nerves,  reflex  augmenta- 
tion (Babinski)  with  spastic  con- 
tractures, changes  in  sensibility 
and  in  the  activities  of  the  sphinc- 
ters; 6.  anatomical  changes  are  found 
regularly  in  the  muscular  apparatus, 
but  in  pure  forms  of  the  disease 
never  in  the  nervous  system. 

The  infantile  atrophic  form 
of  dystrophy  is  relatively  the  most 
frequent;  its  course  may  run  with 
and  more  rarely  without  involve- 
ment of  the  face;  pseudo-hyper- 
trophies are  scarcely  ever  absent. 
The  disease  begins  (it  is  more  fre- 
quent in  boys  than  in  girls)  insidiously  and  very  slowly  in  early  childhood 
between  the  third  and  fifth  years,  after  the  patients  have  already  learned 
to  walk  in  the  normal  way.  The  first  things  the  parents  notice  are:  a 
somewhat  slower,  waddling  and  rocking  gait,  difficulty  in  running,  jump- 
ing and  especially  in  rising  from  a  recumbent  or  bent-over  position.     Simul- 


FiG.  100. — Infantile  atrophic  form  of  dys- 
trophy with  participation  of  the  face.  {After 
Heinr.  Curschmann.) 


426  THE  MYOPATHIES 

taneously,  a  lordosis  of  the  spinal  column  develops.  As  a  rule,  disturbances 
in  the  motility  of  the  upper  extremities  (in  raising  the  arms  and  shoulders) 
are  not  perceived  till  later.  At  this  time  paralytic  phenomena  in  certain 
facial  muscles  also  appear  quite  frequently.  After  having  existed  several 
years  the  disease  reaches  its  climax. 

The  findings  are  now  usually  as  follows  (Fig.  ioo) :  The  most  serious 
disturbances  nearly  always  affect  the  musculature  of  the  trunk,  pelvis  and 
thigh.  The  gait  is  noticeably  uncertain,  rocking,  waddling  (because  of 
the  weakness  of  the  glutaei  medii,  Strumpell),  the  thighs  are  raised  labori- 
ously and  relatively  high,  the  feet  fall  stamping  to  the  ground  (weakness  of 
the  muscles  of  the  thigh).  In  walking  and  standing  a  pronounced  lordosis 
of  the  lumbar  spinal  column  appears,  which  may  attain  grotesque  degrees, 
a  result  of  the  atrophy  of  the  muscles  of  the  back  and  pelvis;  the  abdomen 
protrudes  considerably.  The  atrophies  constantly  single  out,  besides  the 
long  muscles  of  the  back,  the  glutaei,  the  extensors  of  the  leg  proper  (quad- 
riceps), somewhat  less  frequently,  its  flexors.  The  paralysis  of  the  two 
former  causes  the  well-known  phenomenon  in  rising  from  a  recumbent 
position  (cf .  Fig.  102) :  first  the  children  by  means  of  their  arms  raise  them- 
selves high  enough  to  stand  on  "all  fours,"  then  they  climb  up  on  themselves, 
as  it  were,  resting  their  arms  first  upon  their  knees  and  then  upon  their  thighs. 

Associated  with  the  atrophies,  pseudo-hypertrophies  are  nearly  always 
found  in  the  lower  extremities,  most  constantly  in  the  musculis  gastrocnemius; 
less  frequently  traces  of  pseudo-hypertrophy  are  found  in  the  glutsei  and 
the  deltoid.  The  hypei trophic  muscles  usually  feel  more  flaccid  than 
normal  muscles,  often  peculiarly  doughy,  sometimes,  however,  dense  and 
solid.  Their  strength  is  often  noimal,  somewhat  less  frequently  diminished, 
very  rarely  increased. 

In  the  infantile  form  the  muscles  of  the  shoulder-girdle  and  the  arms  are 
for  a  long  time  less  severely  affected  than  those  of  the  trunk  and  the  legs. 
But  atrophies  of  the  m.  pectoralis,  the  m.  cucullares,  the  m.  serrati  and 
others  are  quite  constant.  The  resulting  disturbances  in  motility,  which 
especially  affect  the  raising  and  use  of  the  arms,  when  they  are  lifted  above  a 
horizontal  position,  are  discussed  in  greater  detail  under  the  juvenile  form 
of  the  disease,  in  which  they  play  a  far  greater  part.  The  forearm  is  nearly 
always  spared  in  the  infantile  form. 

The  face  is  attacked  by  the  atrophy  and  paresis  at  an  early  period. 
First  is  noticed  the  weakness  of  the  orbicular  muscles  of  the  eyes  and  the 
mouth;  the  closure  of  the  eyes  is  performed  incompletely  and  lacks  power; 
the  mouth  can  not  be  pursed.  As  a  result  of  atrophy  of  its  muscles,  the 
upper  lip  protrudes  abnormally;  there  develops  a  suggestion  of  "  tapir-snout." 
Later  the  atrophy  extends — not  always  symmetrically — over  the  musculature 
of  the  cheeks  and  forehead,  so  that  finally  the  myopathic  mask-face  appears. 
The  tongue  and  pharynx,  as  well  as  the  muscles  of  mastication — in  contrast 


PSEUDOMUSCULAR  HYPERTROPHY 


427 


to  bulbar  paralyses  of  other  genesis — nearly  always  remain  intact;  likewise 
the  external  and  internal  muscles  of  the  eye. 

It  has  already  been  stated  that  the  sensory  cerebral  nerves,  sensibility 
and  sphincters  remain  normal.  Likewise,  psychic  anomalies  belong  to 
the  exceptions;  on  the  contrary,  the  children,  as  a  rule,  are  intellectually 
alert  and  clever. 

A  rarer  modification  of  infantile  dystrophy  is  pseudo-hypertrophy 
of  the  muscles,  described  first  by  Griesinger,  later  by  Duchenne  and  others. 
It  also  begins  in  earliest  childhood,  between  the  third  and  sixth  years;  its 


Fig.  ioi. — Four  children  of  the  same  parents  with  infantile  muscular  dystrophy.  The  two 
older  children  show  the  atrophic,  the  two  younger  boys,  the  almost  pure  hypertrophic  form. 
{After  Heinr.  Curschmann.) 

appearance  is  very  insidious,  since  the  general  stoutness  of  the  body  does 
not,  at  first,  impress  the  parents,  as  the  sign  of  a  disease. 

In  typical  cases  of  this  disease  the  pseudo-hypertrophies  overshadow 
the  atrophies  decidedly.  The  muscles  of  the  calves  and  thighs,  often  also 
of  the  buttocks,  stand  out  with  enormous  volume;  in  like  manner,  the  muscles 
of  the  shoulder  and  arm  (especially  the  m.  triceps)  become  hypertrophic. 
At  the  same  time  the  abdomen  is  protruded,  because  of  the  existing  lordosis, 
and  seems  abnormally  developed.  The  external  picture  might  resemble 
that  of  a  caricature  of  a  youthful  Hercules. 


428  THE  MYOPATHIES 

At  the  same  time,  the  consistency  of  the  hypertrophied  muscles  is  often 
very  soft  and  spongy,  but  occasionally,  however,  as  in  genuine  hypertrophies, 
solid.  Correspondingly,  the  functioning  power  of  these  muscles  is,  for  the 
most  part,  relatively  reduced;  but  we  have  also  seen  cases  in  which  hyper- 
trophied muscles  were  abnormally  strong.  This  is  true  especially  of  the 
musculature  of  the  calf,  the  endurance  and  strength  of  which  made  one  of 
my  little  patients,  a  virtuoso  in  "toe-running." 

Beside  the  pseudo-hypertrophies,  atrophies  also  are  nearly  always 
discovered  upon  closer  examination;  they  attack  preferentially  the  long 
muscles  of  the  back,  and  not  infrequently  also  the  glutaei.  The  weakness 
of  the  pseudo-hypertrophic  and  atrophic  muscles  of  the  trunk  and  the  legs 
now  cause  disturbances  in  posture  (especially  in  standing  and  walking)  and 
in  the  gait  itself,  which  closely  resemble  those  of  the  atrophic  dystrophies; 
pronounced  lumbar  lordosis  and  waddling,  rocking  outward  walking. 
The  ability  of  the  patient  to  raise  himself  from  the  ground  is  always  dis- 
turbed. The  weakness  of  the  arm  and  shoulder  muscles  appears  less 
distinctly  in  the  pure  pseudo-hypertrophic  forms,  though  it  is  never  alto- 
gether absent.  Atrophy  and  paresis  of  the  musculature  of  the  face  are 
said  not  to  occur  in  these  forms. 

Concerning  the  occurrence  and  course  of  pseudo-hypertrophy  the  fact 
must  be  emphasized,  that  not  infrequently  it  is  found  in  families,  in  which  the 
older  children  suffer  from  atrophic  dystrophy,  as  the  reproduced  dystrophic 
brothers  from  the  Leipsic  clinic  show  in  a  classical  way  (Fig.  101).  In 
such  cases  the  pseudo-hypertrophy  usually  passes  over  into  the  atrophic 
form  with  involvement  of  the  face.  In  other  instances,  also,  transitions 
between  the  two  forms  frequently  occur. 

The  juvenile  form  of  dystrophy,  which  is  almost  as  common  as  the 
infantile  atrophic  form,  does  not  show  familial  appearance  so  frequently 
as  the  forms  just  described.  Sporadic  cases  are  certainly  no  rarities.  The 
disease  attacks,  as  Erb,  Strumpell  and  I  have  observed,  the  female  sex 
relatively  more  frequently  than  do  the  infantile  forms. 

The  onset  in  the  juvenile  form  occurs  about  the  middle  of  the  second 
decade  and  is  usually  most  insidious,  even  though  more  rapid  progress 
has  supposedly  been  observed  after  traumatism. 

As  characteristic  distinctive  signs  from  the  forms  discussed  heretofore, 
we  must  mention  first  and  foremost:  the  prevalence  and  the  early  beginning 
of  the  disturbances  in  the  shoulder- girdle  and  the  upper  extremities  and  the 
falling  into  the  background  of  the  pseudo -hyper trophy  in  the  juvenile  form. 
It  is  not  improbable  that  the  former  characteristic,  lacking  in  the  infantile 
form,  is  to  be  explained  by  the  considerably  increased  demands  that  are 
being  made  upon  the  muscles  of  the  arms  and  shoulders  (in  the  sense  of  the 
"using  up"  theory  of  L.  Edinger). 

The  entire  type  of  the  patients  differs  from  that  of  the  infantile  form  by 


PROGRESSIVE  MUSCULAR  DYSTROPHY 


429 


FlG.  102. — a-e.  Rising  from  the  ground  in  dystro- 
phia musculorum.     {After  Heinr.  Curschmann.) 


Fig.  102,  b. 


Fig.  102,  c. 


Fig.  102,  d. 


Fig.  102,  e. 


43°  THE  MYOPATHIES 

the  pronounced  changes  in  the  form  of  the  shoulders,  thorax  and  arms 
(Fig.  103).  Owing  to  the  paralyses  of  the  m.  cucullares,  of  the  muscles  that 
fix  the  scapula  and  in  part  of  the  m.  pectorales,  the  shoulders  sink  far  down- 
wards and  frequently  not  only  downwards  but  forwards  as  well.  From  these 
"fallen"  shoulders,  arms  hang  often  thin  as  broom-sticks,  and  at  their  top 
lies  a  deltoideus  of  almost  normal  volume  which  has  also  slipped  downwards 
and  forearms,  which,  by  atrophy  of  the  supinator  longus  and  occasionally 
of  other  muscles,  have  lost  their  oval  contour  and  acquired  an  approximately 
round  one.  Through  the  overhanging  of  the  shoulders  and  the  atrophy 
of  the  pectoral  muscles,  which  causes  the  breast-arm  furrow  to  run  upwards 
instead  of  downwards,  there  appears  a  sunken  breast  form,  the  "thorax  en 
bateau,"  occasionally  an  actual  funnel-shaped  thorax.  Especially  striking 
are  the  changes  in  form  and  disturbances  in  function,  which  are  produced 
through  the  defects  of  the  scapular  muscles.  Through  paralysis  of  the  ser- 
ratus.  the  scapulae  protrude  like  wings,  not  only  while  at  rest,  but  especially 
when  lifting;  in  addition,  through  the  disappearance  of  the  m.  cucullares, 
the  shoulder-blades  are  in  an  abnormally  low  position  and  far  from  the  mid- 
dle line.  If  the  attempt  is  made  to  raise  the  patient  by  or  under  the  upper 
arms,  this  fails,  as  the  shoulders  are  "loose, "  cannot  be  fixed,  and  during  the 
attempt  are  pulled  limply  upwards  above  the  ears.  If  the  patient  is  ordered 
to  press  the  arm,  that  has  been  raised  to  a  horizontal  position,  downwards 
against  a  resistance,  there  also  appears  a  characteristic  phenomenon: 
because  of  the  disappearance  of  the  m.  latissimus  dorsi  the  attempt  men- 
tioned fails  and  the  unfixed  shoulder-blades  are  now  partly  actively,  partly 
passively  and  indirectly  pushed  back  and  down  (by  the  resistance). 

Beside  this  the  appearance  of  the  juvenile  dystrophic  patient  is  charac- 
terized by  dorso-lumbar  lordosis  and  the  secondarily  protuberant  abdomen 
(Fig.  104).  The  gait  also,  because  of  the  atrophy  of  the  glutaei  and  some 
of  the  muscles  of  the  thigh,  is  waddling  and  rocking,  even  though  fre- 
quently less  so  than  in  the  infantile  forms. 

To  recapitulate  briefly  the  following  muscles  are  usually  atrophic: 
cucullaris,  pectoralis  major  and  minor,  serratus  anticus,  latissimus  dorsi,  the 
long  muscles  of  the  back  and  loins,  somewhat  less  regularly  and  later,  rhom- 
boidei  and  triceps,  as  well  as  the  supinator  longus.  The  following  are,  as  a 
rule,  spared:  sternocleido-mastoideus,  levator  anguli  scapulas,  coracobrachia- 
lis,  teres  major  and  minor,  supra-  and  infra-spinatus,  the  flexors  and  exten- 
sors of  the  forearm  and  the  small  muscles  of  the  hand.  In  the  lower  extrem- 
ities the  following  are  nearly  always  atrophic:  m.  quadriceps  and  the  glutaei, 
somewhat  less  frequently  the  flexors  of  the  thigh  (m.  biceps,  semitendinosus, 
etc.),  and  the  adductors,  very  rarely  the  m.  tibialis  anticus,  the  peronei  and 
the  extensors  of  the  toes.  The  calf  musculature  (in  respect  to  function) 
mostly  remains  normal,  but  very  frequently  it  shows  signs  of  pseudo-hyper- 


MORBID  ANATOMY  OF  MUSCULAR  DYSTROPHY 


431 


trophy.     In  the  upper  half  of  the  body  the  m.  supra-  and  infra-spinati  and 
the  m.  triceps  are  not  infrequently  hypertrophic. 

The  mechanical  excitability  of  the  atrophic  muscles  (general  and  idio- 
muscular)  shows  a  simple  diminution,  or  it  may  be  lost.  Likewise,  electrical 
tests  show  only  a  quantitative  change  up  to  the  loss  of  the  reaction;  in  a  few 
cases,  a  suggestion  of  R.D.  is  said  to  have  been  observed.  The  tendon 
reflexes  usually  disappear  in  the  regions  that  are  affected;  frequently,  also, 
I  have  found  general  abolishment  of 
the  tendon  and  periosteal  reflexes. 
The  cutaneous  reflexes  remain  intact. 

Anatomy. — Brain,  spinal  cord, 
and  peripheral  nervous  system  have 
(in  pure  cases)  been  found  normal 
in  all  cases  examined  thus  far,  even 
by  the  most  delicate  histological 
methods. 

The  muscles,  on  the  other  hand, 
always  show  striking  changes:  in 
the  atrophic  muscles  numerous  mus- 
cular fasciculi  are  completely  de- 
stroyed and  their  spaces  filled  up  by 
the  infiltration  of  fat;  the  greater 
number  of  the  individual  muscular 
fibres  are  markedly  atrophic,  but  a 
part  of  them  are  also  of  abnormally 
large  circumference;  the  muscle  nu- 
clei are  increased,  the  muscular  fibres 
themselves  usually  show  fissures  and 
vacuoles,  beside  proliferation  of  the 
interstitial  connective  tissue.  While 
in  pseudo-hypertrophic  muscles,  al- 


Fig.  103. — Juvenile  form  of  dystrophy. 
Atrophy  of  the  face  (lips),  pronounced  atrophy 
of  the  m.  cucullares,  serrati  (-wing  position  of 
the  shoulder-blades)  "sunk  in  shoulders"  and 
atrophy  of  the  muscles  of  the  upper  arm. 
(Heidelberg  Medical  Clinic.) 


though  the  atrophy,  the  fatty  degeneration  and  proliferation  predominate, 
still  there  are  muscles  which,  owing  to  an  extraordinary  increase  in  volume  in 
the  original  fibres,  are  to  be  considered  genuinely  hypertrophic. 

^Etiology. — An  actual  "exciting"  cause  of  dystrophy  is  not  known. 
That  traumatism  could  fill  this  position  I  consider  very  doubtful.  The 
disease  is  to  be  interpreted  rather,  as  the  expression  and  consequence  of  a 
specifically  weak  congenital  predisposition  of  the  muscular  system,  which 
succumbs  to  use,  sooner  or  later,  in  this  typical  way. 

Course  and  Prognosis. — The  course  is  always  very  chronic;  whereas 
the  infantile  patients  rarely  reach  adult  life,  dying,  as  a  rule  early,  of  inter- 
current diseases;  the  juvenile  form  lasts  a  long  time.  There  are  not  a  few 
cases  in  which,  dystrophy  notwithstanding,  the  50th  and  60th  years  was 


432  THE  MYOPATHIES 

reached.  The  progress  is  usually  very  slow;  especially  juvenile  cases  fre- 
quently show  a  standstill  of  the  disease  for  ten  years  and  more.  The  prog- 
nosis quoad  valetudinem  is  always  bad.  I  know  of  only  one  case  through 
(verbal)  communication  of  Erb,  in  which  dystrophy,  so  diagnosed  by  him,  in 
a  little  girl,  resulted  in  recovery. 

Treatment  is  correspondingly  almost  always  powerless,  even  though 
mild  hydriatic,  gymnastic  and  electric  procedures  are  occasionally  supposed 
to  have  caused  improvement  (in  connection  with  the  rest  of  the  hospital 
treatment).  The  organ- therapeutic  preparations  have  proved  to  be  ineffec- 
tual. In  some  cases  (especially  in  the  infrequent  complications  with  con- 
tractures) orthopedic  surgical  interference  may  be  indicated.  Supporting 
corsets,  on  the  whole,  have  not  proved  very  serviceable. 

Differential  diagnosis  must  consider  in  the  infantile  form,  the  similarity 
— which,  however,  is  quite  superficial — to  diplegia  infantum;  this,  however, 
may  be  immediately  distinguished  by  its  pyramidal  tract  symptoms.  Polio- 
myelitis anterior  could  but  rarely  be  confused  with  dystrophy.  In  the  pseudo- 
hypertrophic form  a  possible  confusion  with  myotonia  congenita  must  be 
thought  of;  however,  the  characteristic  disturbance  in  motility  and  muscular 
reaction  of  the  latter  should  at  any  rate  easily  establish  the  diagnosis.  Dif- 
ferentiation from  Thomsen's  disease  with  muscular  wasting  may  be 
more  difficult,  since  the  myoatrophy  here  occasionally  shows  the  typical 
distribution  of  dystrophy.  But  even  in  such  cases  the  existence  of 
myotonic  reaction  of  individual  muscles  will  decide  the  differential  diagnosis. 
Furthermore,  the  rare  cases  of  actual  genuine  muscular  hypertrophy  (either 
congenital  or  acquired)  may  appear  in  differential  diagnostic  competition, 
and  also  the  very  rare  cases  of  hypertrophy  following  thrombosis,  neuritis 
and  in  spastic  spinal  paralysis.  A  consideration  of  the  manner  of  the  dis- 
tribution of  the  muscular  dystrophy  will  make  the  diagnosis  in  all  these  cases 
easy.  The  not  uncommon  cases  of  congenital  defects  of  single  muscles 
(especially  of  the  trapezius  and  pectoral  muscles)  may  also  arouse  a  slight 
suspicion  of  dystrophy. 

Most  important  in  differential  diagnosis  are,  naturally,  the  other  forms  of 
muscular  wasting:  the  spinal,  the  neural  (Marie- Hoffmann' 's)  amyotrophy, 
syringomyelia,  the  muscular  atrophies  in  polyneuritis  and  the  already  men- 
tioned myotonia  amyotrophica.  The  first  two  forms  mentioned  are  imme- 
diately distinguished  from  dystrophy,  by  the  fact  that  they  show  an  opposite 
arrangement  of  atrophies :  the  preference  for  the  distal  ends  of  the  extremities, 
whereas  dystrophy  shows  preference  for  the  proximal  parts.  In  rare  cases, 
of  course,  one  must  reckon  with  the  beginning  of  the  spinal  affection  in  the 
muscles  of  the  back.  The  same  thing  holds  true  of  the  polyneuritic  forms  of 
muscular  wasting.  Syringomyelia  may  indeed,  even  if  rarely,  exhibit  its 
earliest  atrophy  in  the  proximal  arm  muscles  and  the  shoulder- girdle;  but 
in  this  case  the  disturbance  in  sensation  will  immediately  decide  the  differ- 


RARE  FORMS  OF  MUSCULAR  DYSTROPHY 


433 


ential  diagnosis.  But  above  all,  in  all  the  before-  mentioned  conditions,  the 
appearance  of  fibrillary  tremors  and  of  the  electrical  reaction  of  degeneration 
make  the  diagnosis  considerably  easier. 

One  must  mention,  finally,  that  occasionally  inflammatory  diseases  of 


Fig. 


104,  a    and    b. — Lordosis   and   typical   gait   in   juvenile   dystrophy.     {After  Heinr. 

Curschmann.) 


the  muscles  with  atrophy  (Oppenheim)  and  certain  diseases  of  the  bones 
(e.  g.,  rachitis  tarda  (author))  may  remind  superficially  of  dystrophy. 

Rare  Forms  and  Complications. — Here,  first  and  foremost,  the  form 
observed  by  /.  Hoffmann,  with  bulbar  paralytic  localization  of  dystrophy, 
must  be  mentioned.     Also  by  Oppenheim  and  others,  the  (very  rare)  par- 
28 


434  THE  MYOPATHIES 

ticipation  of  the  muscles  of  the  tongue  and  pharynx  has  been  established; 
likewise  the  spread  of  the  disturbance  to  the  muscles  of  the  eye.  That  the 
dystrophy  may  begin,  quite  exceptionally,  in  the  legs  proper,  in  the  hands 
and  forearms,  was  proved  by  the  reports  of  /.  Hoffmann,  Schultze  and  Erb. 
Furthermore  those  cases  are  remarkable,  in  which  the  trophic  changes  are 
not  confined  to  the  musculature,  but  extend  also  to  the  bones  {Friedreich,  E. 
Schultze,  Schlippe  and  others) ;  some  of  these  cases  show  in  addition  extensive 
early  contractures  in  the  muscles.  Anomalies  in  the  formation  of  the  skull 
and  thorax  have  also  been  observed. 

We  must  mention,  furthermore,  that  dystrophy  has  been  observed  in 
combination  with  all  sorts  of  organic  and  functional  nervous  disease  (for 
instance,  with  tabes,  epilepsy,  hysteria  or  psychoses).  It  is  probable  from 
late  observations,  that  there  are  forms  of  dystrophy,  which  never  attain 
complete  development  (formes  frustes). 

2.  MYOTONIA  CONGENITA,  THOMSEN'S  DISEASE] 

This  rare  disease,  described  first  by  Ch.  Bell  and  Leyden,  but  in  the  year 
1876,  actually  "discovered"  by  the  Silesian  physician  Thomsen  and  classic- 
ally worked  up  by  Erb  must  still  be  regarded — like  progressive  dystrophia 
musculorum — so  long  as  no  stringent  proof  to  the  contrary  be  adduced,  as  a 
primary  myopathy,  as  a  severe  intention  neurosis  of  the  muscles. 

The  disease,  which  in  many  cases,  especially  in  the  family  of  the  dis- 
coverer, appears  mostly  familial,  with  a  preference  for  males,  begins  in  the 
typical  cases  usually  in  early  youth,  and  not  seldom,  too,  about  the  time  of 
puberty.  It  usually  progresses  very  slowly,  rapidly  only  upon  the  occurrence 
of  unusually  strong  physical  exertion  (military  service)  and  is  then  occasion- 
ally heightened  to  an  acute  onset.  The  first  symptoms  are  nearly  always  a 
certain  stiffness  and  difficulty  in  walking,  rising,  lifting,  etc.,  after  a  period 
of  rest,  especially  in  the  morning.  This  stiffness  diminishes  upon  the  repeti- 
tion of  the  movement  concerned  and  finally  disappears  altogether. 

The  typical  picture  of  the  completely  developed  myotonia,  is  as  follows: 
the  young  men,  who  are  usually  well-nourished  and  not  seldom  full-blooded, 
show  an  almost  athletic  development  of  the  muscles  of  the  trunk  and  the 
extremities,  which  contrasts  strikingly  with  the  functional  weakness  of 
which  they  complain.  At  every  first  attempt  at  movement  in  any  group  of 
muscles,  after  they  have  been  rested  (especially  in  the  extremities),  there 
is  a  peculiar  tension  and  stiffness,  a  veritable  spasm  of  the  muscles  concerned, 
which  causes  a  marked  slowing  of  the  movement;  and  this  "myotonus" 
usually  reaches  its  height  at  the  second  movement  (not  the  first) ;  for  instance, 
if  the  first  adduction  of  the  thumb  succeeds  with  relative  speed,  the  follow- 
ing abduction  may  meet  the  strongest  resistance.  This  difference  between 
the  first  and  second  movement  is  exhibited  very  distinctly  in  rapidly 
executed  powerful  movements. 


MYOTONIA  CONGENITA  435 

Upon  further  repetition  of  the  movement  concerned,  it  becomes  freer 
and  freer,  the  tension  is  soon  relaxed  and  the  movement  becomes  absolutely 
normal.  The  disturbance  in  the  gait  appears  in  a  most  characteristic  form: 
at  the  beginning  slow,  short,  dragging  steps  (veritably  spastic)  are  taken 
with  great  exertion- — after  8-10  m.  the  walk  is  freer  and  becomes  completely 
normal,  quick  and  elastic  at  the  end  of  a  walk  of  about  15  m.  (for  instance, 
a  walk  through  a  hospital  ward).  The  disturbance  becomes  very  serious, 
if  the  patient  is  suddenly  forced  to  make  a  brusque  and  quick  movement; 
for  instance,  to  avoid  an  obstacle  that  appears  in  his  path,  or  the  like;  the 
legs  may  then  become  as  stiff  as  sticks,  the  patient  falls  down  like  a  log, 
and  can  only  rise  with  difficulty.  The  myotonic  disturbance  also,  is  usually 
increased  by  excitement  and  by  cold. 

This  disturbance  may  now — in  rarer  cases — be  restricted  to  definite 
muscle  areas;  for  instance,  to  the  legs,  hands,  tongue,  etc.;  but  mostly,  it 
appears  generalized,  attacking  all  the  muscles  of  the  trunk  and  limbs,  even 
if  not  with  the  same  severity.  It  often  disturbs  even  the  motility  of  the 
muscles  of  the  face  and  of  mastication,  of  the  muscles  of  the  tongue  and  of 
those  that  close  the  eyelid,  very  rarely  those  of  the  throat  and  oesophagus 
and  the  external  muscles  of  the  eye;  it  is  doubtful  whether  the  musculature 
of  respiration  and  the  heart  ever  participate. 

The  affected  muscles  do  not  usually  show  an  increase  in  tonicity  while 
at  rest  and  during  passive  movements;  the  passive  movements  are  completely 
free,  in  contrast  to  the  active  ones;  the  same  is  true  of  the  reflex  movements 
(for  instance  the  plantar  reflex).  On  the  other  hand,  the  mechanical  and 
electrical  excitability,  as  Erb  first  showed,  are  changed  in  a  most  striking  way. 

The  mechanical  excitability  is  usually  perceptibly  augmented;  every 
tapping  of  the  muscle,  often  mere  pressure  or  kneading,  produces  an  abnormal 
continuation  of  the  local  muscular  contraction  for  from  5  to  30  seconds; 
there  arise,  according  to  the  location  and  form  of  the  muscles,  either  weals 
or  deep  grooves  and  furrows  (with  special  distinctness  in  the  tongue,  the 
m.  gastrocnemius,  etc.).  At  the  same  time,  the  idiomuscular  excitability 
is  very  slight;  Schiff's  waves  are  also  absent. 

The  electrical  excitability  is  usually  absolutely  increased  for  both  kinds 
of  currents.  With  the  usual  faradic  (direct)  stimulation,  employing  some- 
what stronger  currents,  there  is  occasionally  a  somewhat  slow  contraction 
with  long  prolongation  (2  to  20  seconds  or  more) ;  single  opening  shocks  of 
any  strength  whatever  cause  only  normal,  quick  contraction;  with  strong 
stabile  faradization,  there  appear,  occasionally,  oscillating  muscular  waves. 
The  contractions  caused  by  (direct)  galvanization  also  are  slow,  tonic,  and 
show  abnormal  prolongations;  usually  only  closing  contractions  (ACC  = 
CCC)  appear;  in  stabile  galvanization,  occasionally  (not  always  and  not  in 
all  muscles)  rhythmical  wave-like  contractions  from  the  C  to  the  A.  This 
electric  symptom  complex  is  named,  according  to  Erb,  "  myotonic  reaction." 


436  THE  MYOPATHIES 

The  nerve  trunks  usually  do  not  show  any  peculiar  change  in  excitability 
upon  mechanical  and  electrical  stimulation;  with  indirect  electric  stimulation, 
therefore,  there  is  no  myotonus. 

The  sensory  functions,  the  sphincters,  the  sensibility  and  the  cutaneous 
reflexes  have  always  been  found  normal ;  the  tendon  reflexes  were  occasionally 
weakened,  or  abnormally  easily  wearied,  but,  as  a  rule,  active.  Trophic 
and  vaso-motor  disturbances  are  nearly  always  absent.  The  psyche  is 
usually  normal — which  must  be  emphasized  as  against  the  classification 
of  the  disease  under  the  psychoses  by  Thomsen  himself — even  though  a 
combination  with  hysteria  and  epilepsy  has  been  observed. 

Varieties  of  Myotonia. — The  most  important  variety  is  the  one  accom- 
panied by  different  forms  of  muscular  wasting,  amyotrophic  myotonia, 
which  has  been  observed  rather  frequently  in  recent  times — in  about  20 
to  25  cases.  In  contrast  to  typical  myotonia,  it  does  not  appear  to  begin 
in  early  youth,  but  not  until  the  second  to  the  third  decade,  to  run  its  course 
simultaneously  with  atrophy  and  pareses  and  myotonic  disturbances. 
The  myotonia  may  remain  more  or  less  generalized,  but  may  also  limit 
itself  to  only  a  few  muscles  (especially  to  the  hands  and  tongue).  The 
atrophies  may  be  of  the  type  of  ErVs  dystrophy,  now  but  more  rarely,  of 
that  of  the  Duchenne-Aran  amyotrophy  (forearm-peroneal  type);  atrophy 
of  the  muscles  of  the  face  and  mastication  is  relatively  frequent  (spontaneous 
dislocation  of  the  mandibula),  as  well  as  speech  disturbances,  but  ptosis 
(Fiirnrohr)  is  very  rare.  Once  inceptive  atrophies  were  proved  present  in  a 
previously  typical  case  of  congenital  myotonia  (Hoffmann).  The  myotonic 
reaction  is,  of  course,  modified  by  the  atrophies;  occasionally  one  finds  the 
myasthenic  reaction.  The  tendon  reflexes  are  not  rarely  entirely  absent 
in  the  amyotrophic  myotonia;  sometimes  there  is  mechanical  over-excitability 
of  the  nerves.  The  course,  because  of  the  atrophies,  is  more  severe,  but 
improvements  are  possible. 

The  combination  of  syringomyelia  with  myotonia,  which  has  been 
observed  in  some  cases,  is  also  to  be  placed  in  the  domain  of  amyotrophic 
myotonia. 

Myotonia  acquisita  has  been  described  by  Talma  in  cases  in  connection 
with  traumatisms  and  acute  infections.  The  prognosis  in  it  seems  more 
favorable  than  in  idiopathic  cases. 

Furthermore,  in  very  rare  cases,  there  have  been  observed  instances  of 
intermittent  myotonia;  myotonia-like  neuroses,  accompanied  by  intermittent 
paralyses  (paramyotonia,  Eulenburg),  of  myotonia  combined  with  tetany 
(Bettmann,  v.  Voss),  of  myotonia  of  nursing  infants  and  of  those  suffering 
with  gastric  dilatation. 

The  differential  diagnosis  of  generalized  myotonia  scarcely  ever  pre- 
sents difficulties;  at  most  some  cases  of  pseudo-hypertrophic  dystrophia  mus- 
culorum or  rare  cases  of  spastic  spinal  paralysis  may  display  a  superficial 


MYASTHENIA  PSEUDOPARALYTIC  A  437 

similarity  to  it;  the  correct  diagnosis,  however,  is  easily  recognized  by  the 
demonstration  of  the  typical  disappearance  of  the  myotonus  after  repetition 
of  the  movement  and  by  the  myotonic  reaction.  The  partial  myotonia,  for 
instance,  of  the  forearms,  may  bear  a  slight  resemblance  to  the  chronic  tonic 
form  of  tetany  and  to  occupational  cramps,  without,  however,  causing 
serious  differential  diagnostic  difficulties.  The  pseudo-spastic  paresis  of 
hysteria  may  also— very  rarely  and  only  superficially — resemble  myotonia. 
In  the  amyotrophic  form,  however,  the  separation  from  other  forms  of 
muscular  wasting,  sometimes  succeeds  only  after  an  exact  electrical  investi- 
gation, especially  of  the  non-atrophic  musculature. 

Morbid  Anatomy. — So  far,  the  central  nervous  system  has  been  found  to 
be  free  from  all  changes.  On  the  other  hand,  the  muscles,  according  to  Erb, 
show  the  following  changes:  enormous  hypertrophy  of  all  the  fibres,  with 
most  profuse  proliferation  of  the  nuclei  of  the  sarcolemma,  changes  in  the 
finer  structure  (indistinct  striation,  formation  of  vacuoles,  etc.),  besides  a 
slight  increase  of  the  interstitial  connective  tissue  and  deposit  of  a  granular 
substance.  Schieffer decker  recently  proved  that  the  nuclear  increase  is  only 
relative  and  that  in  the  sarcoplasma,  peculiar  grains  are  found,  when  a 
certain  fixing  agent  is  employed. 

The  actual  nature  of  the  disease  is  not  yet  clear;  the  heretofore  assumed 
purely  myogenous  theory  is  not  wholly  satisfactory,  in  spite  of  Schieffer- 
decker's  findings.  Several  symptoms  point  perhaps  to  a  central  (cerebral?) 
source  of  the  disease. 

Correspondingly,  the  aetiology  of  myotonia  congenita  has  not  been  at 
all  explained;  the  thought  of  an  auto-intoxication  (analogy  with  veratrin 
poisoning)  has  only  hypothetical  interest.  The  important  role  of  heredity 
speaks  for  a  congenital  failure  in  development  of  some  kind. 

The  prognosis  of  typical  Thomserts  disease  is  quoad  valetudinem 
always  bad,  quoad  vitam  always  good.  The  patients  are  mostly  unsuited  to 
vocations  which  are  physically  exhausting.  The  varieties,  especially  amyo- 
trophic myotonia,  the  myotonia  of  gastric  dilatation  and  of  babies  at  the 
breast  have  a  more  serious  prognosis. 

Treatment  is — in  typical  cases — always  fruitless;  nerve-stretchings 
have  not  proved  to  be  of  any  value.  Careful  gymnastics  and  massage  are 
supposed  to  be  able  to  induce  functional  improvements.  The  main  point 
seems  to  be  prophylaxis  with  regard  to  accidents. 

3.  MYASTHENIA  PSEUDOPARALYTICA 

{Myasthenic  Bulbar  Paralysis,  ErVs  Disease) 
The  first  communications  concerning  this  peculiar  disease,  we  owe  to 
Erb;  later  the  investigations  of  Oppenheim,  Hoppe,  Goldflam,  Strumpell, 
Eisenlohr,  Jolly  and  others  have  taught  us  more  of  the  nature  of  the  disease 
and  clinically,  at  least,  they  have  cleared  the  subject  fully. 


438  THE  MYOPATHIES 

We  must  regard  the  peculiarity  of  myasthenic  paralysis  to  lie  in  the  fact, 
that  it  leads  to  serious  paralyses  of  the  bulbar  nervous  realms,  first  and  foremost 
of  the  muscles  of  the  eyes  and  the  pharynx,  furthermore,  to  paralytic-like  weakness 
of  the  musculature  of  the  trunk  and  the  extremities,  without  ever  attaining  degen- 
erative atrophies  or  hypertonic  muscular  changes,  while  the  macroscopic  or 
microscopic  findings  in  the  central  nervous  system  do  not  show  sufficient  changes 
to  explain  these  disturbances.  The  paralyses  in  all  the  regions  have,  as 
Gold-flam  first  showed,  especially  in  the  beginning,  a  clear  remittent  character; 
they  are  the  product  of  a  morbidly  increased  capacity  for  fatigue.  The 
prognosis  is  mostly  unfavorable;  recovery  is,  on  the  whole,  rare. 

Course  and  Symptomatology. — The  disease  attacks  individuals  who 
have  been  well  heretofore,  between  the  20th  and  the  40th  years;  women  are 
affected  somewhat  more  frequently  than  men.  Usually  very  gradually,  more 
rarely  rapidly  after  an  acute  disease,  after  traumatism  or  over-exertion,  the 
patients  become  afflicted  with  paralytic  phenomena  of  the  muscles  of  the  eye, 
very  often  with  ptosis  and  diplopia;  at  the  same  time  one  or  the  other  bulbar 
function,  phonation  or  deglutition  suffers.  These  phenomena  may  also  be 
associated  with  a  feeling  of  weakness  in  the  nape  of  the  neck,  the  trunk  and 
extremities,  even  though  in  the  beginning  the  bulbar  symptoms — they  are 
often  the  only  symptoms  for  years — predominate.  All  disturbances  may 
then  disappear  for  weeks,  months  or  years,  without  leaving  a  trace,  so  that 
the  disease  at  the  beginning  may  consist  of  short  exacerbations  between  long 
remissions.  This  relatively  harmless  initial  stage  may  persist  for  a  long 
time,  up  to  22  years  {author).  Occasionally,  however,  the  course  of  the 
disease  is  more  rapid  and  approaches  its  climax  in  weeks  and  months 
gradually,  without  longer  periods  of  improvement. 

It  is  precisely  in  the  first  stage,  that  this  disease  shows  its  characteristic 
trait,  one  that  distinguishes  it  totally  from  all  other  bulbar  symptom-com- 
plexes, namely,  the  symptom  of  paralysis,  through  pathologically  increased 
fatigue:  the  muscles  of  the  eye,  the  muscles  of  deglutition,  which  in  the  morn- 
ing, after  the  strengthening  rest  of  the  night,  perform  their  functions  well  or 
fairly  well,  fail  after  a  shorter  or  longer  activity,  more  and  more,  in  the  course 
of  the  day,  until  they  become  paralytic  (or  more  exactly,  pseudo-paralytic). 
A  night's  rest  or  some  hours  of  repose  during  the  day  suffice  to  remove  the 
paralysis  and  the  next  day  the  drama  of  myasthenic  fatigue  paralysis  begins 
anew.  Once  the  disease  reaches  its  climax  or  more  particularly,  if  it  reaches 
the  final  stage,  part  of  the  paralyses,  however,  persist,  or  show  only  slight 
changes  according  to  the  condition  of  rest  or  fatigue. 

Gradually,  often  too,  suddenly,  the  climax  of  the  disease  is  reached:  the 
eye  muscles,  the  muscles  of  mastication  and  of  deglutition,  more  rarely 
those  of  the  facial  region,  frequently  the  muscles  of  the  nape  of  the  neck  and 
of  the  back  become  almost  permanently  paretic;  the  fatigue  paralysis  of  the 
extremities  reaches  so  high  a  degree,  that  the  patients  become  permanently 


MYASTHENIA  PSEUDOPARALYTICA 


439 


bedridden.  Nutrition  suffers  through  the  deglutition  paralysis;  faulty- 
swallowing  appears;  finally,  too,  the  musculature  of  respiration  fails  (pos- 
sibly also  the  muscle  of  the  heart)  and  the  end,  prepared  by  inanition,  appears 
quite  spontaneously  as  the  result  of  respiratory  or  cardiac  paralysis,  or 
suddenly  after,  an  action  that  strains  the  heart  (stomach  pump,  different 
baths)  or  is  more  slowly  brought  about  by  an  aspiration  pneumonia. 

Rarely  the  myasthenia  runs  its  course  without  actual  bulbar  paralysis, 
as  pure  paresis  of  the  extremities  and  the  trunk  (Grund,  Rautenberg  and 
others). 

Of  the  objective  symptoms  of  the  completely  developed  disease  the 
disturbances  of  the  muscles  of  the  eye  appear  most  prominently  in  the  fore- 


FiG.  105. — Myasthenia' with  ophthalmoplegia  ex- 
terna on  the  right  side.  {After  Knoblauch.) 


Fig.    106. — The  same  patient   trying   to 
look  upwards.     {After  Knoblauch.) 


ground;  the  ptosis,  which  the  patients  attempt  in  vain  to  counteract  by  auxil- 
iary contraction  of  the  muscles  of  the  forehead,  the  paralyses  of  the  n. 
abducentes,  of  the  oculomotor  muscles  and  the  rest,  so  that  a  total  ophthal- 
moplegia externa  results.  The  defects  in  mobility  frequently  seem  to  be  of 
strictly  associated  nature  (Bielschowsky) ;  it  is  characteristic  that  the  sphincter 
iridis  only  rarely  is  involved  in  fatigue  paralysis.  Frequently  there  exists — 
in  incomplete  paralysis  of  the  muscles  of  the  eye — diplopia  in  a  degree  that 
varies  from  morning  to  night. 

The  muscles  of  mastication  become  paretic  markedly  and  very  early  in 
the  disturbance;  after  a  few  bites  the  masseters  and  temporals  give  out; 
swallowing  is  disturbed  by  the  increasing  paralysis  of  the  soft  palate,  of  the 
muscles  of  the  pharynx  and  oesophagus;  liquid  nourishment  flows  out  through 


44© 


THE  MYOPATHIES 


the  nose.     The  laryngeal  muscles,  mostly  the  phonatores,  rarely  then, 
postici,  fail,  the  voice  becomes  hoarse,  aphonic. 

The  muscles  of  the  lips,  the  naso-labial  muscles  (most  rarely  the  m. 
frontales),  finally  almost  the  entire  facial  region  is  paralyzed;  the  patient  has 
a  worried,  tired,  "mask"  face. 

At  the  height  of  the  disease,  as  we  have  said,  the  muscles  of  the  nape  of 
the  neck  and  all  the  muscles  of  the  trunk  show  signs  of  weakness  and  fatigue; 
arms  and  legs,  especially  in  their  proximal  muscular  regions,  become  tired 
and  paretic  after  a  few  movements,  after  a  little  lifting  and  bending,  after  a 
few  steps. 


Fig.  107. — Forty-five-year-old  patient  with  myasthenia,  bilateral  ptosis,  myopathic  mask-face. 

{Author's  own  observation.) 

At  the  same  time,  the  paralyzed  muscles  (in  pure  cases  of  the  disease) 
never  present  atrophies,  fibrillary  twitchings,  electric  or  mechanical  reaction  of 
degeneration. 

On  the  other  hand,  one  finds  in  many  muscles  (but  by  no  means  always 
in  those  that  are  permanently  paretic  and  most  seriously  affected)  besides  the 
active  overcapacity  for  fatigue,  also  the  same  thing  for  the  faradic  current,  the 
myasthenic  reaction  discovered  by  Jolly  (Mya.  R.)  (Fig.  108) ;  if  such  a  muscle 
is  stimulated  by  direct  tetanization,  it  reacts  at  first  with  normal  contractions, 
gradually  the  closing  contractions  lose  their  intensity  and  finally  fail  alto- 
gether; after  the  tetanization  has  been  stopped  for  a  short  while  (minimum 
ca.  2  sec.)  the  muscle  is  again  capable  of  renewed  contraction.  The  myas- 
thenic reaction  does  not  need  to  be  complete,  but  it  is  at  least  suggested,  in 


MYASTHENIA  PSEUDOPARALYTIC  A 


441 


most  of  the  cases.  The  degree  and  rapidity  with  which  the  Mya.  R.  is 
elicited,  seems  to  stand  in  direct  proportion  to  the  former  voluntary  motor 
or  even  electrical  fatigue  of  the  muscle.  ^ 

It  must  be  mentioned  that  the  Mya.  R. 
has  been  found  also  in  the  atrophic  muscles 
in  other  nervous  diseases,  in  focal  injuries 
of  the  brain,  in  "cerebral  muscular  atro- 
phies" (H.  Steinert),  in  amyotrophic  myo- 
tonia, etc.  These  findings,  however,  can 
not  cast  doubt  on  the  specific  nature  of  the 
reaction  in  the  myasthenia,  which  runs  its 
course  without  atrophies. 

The  sensory  cerebral  nerves  (optic,  ol- 
factory, glosso-pharyngeal,  auditory,  etc.) 
are  always  spared  by  the  disease.  The  sen- 
sory functions  of  every  other  sort  also 
always  remain  intact,  usually,  too,  the 
bladder  and  rectum. 

The  tendon  reflexes  remain  mostly  nor- 
mal; in  rare  cases  they  are  weakened;  often, 
if  frequently  elicited,  they  become  over- 
fatigued  and  disappear.  Rarely  they  may 
be  permanently  lost  during  the  period  of 
total  paralysis  (author's  observation).  The 
cutaneous  reflexes  always  remain  normal. 

The  psyche  of  the  patient  is  mostly 
intact,  always,  too,  the  higher  functions 
of  speech.  Compulsory  emotions,  the  crux 
of  the  atrophic  and  pseudo-bulbar  para- 
lytics, seem  to  be  absent. 

Morbid  Anatomy. — The  brain,  spinal 
cord  and  efferent  nerves  have  been  found 
altogether  intact,  in  most  typical  cases,  ex- 
cluding a  few  in  which  the  changes  were 
insufficient  to  explain  the  serious  dis- 
turbances. On  the  other  hand,  in  numer- 
ous cases,  accumulations  of  lymphoid  cells  fe 
have  been  found  in  the  affected  muscles  (Weigert,  Flatau,  Link,  Goldflam, 
Laqueur,  Boldt,  Burr,  author  and  others) ,  which,  because  of  their  frequency  in 
the  more  recent  postmortem  examinations,  possibly  have  a  pathognomonic 
importance.  In  one  case  fatty  transformation  of  the  sarcoplasm  was  found 
(Marburg).  In  several  cases  persistent  thymus  or  thymus  tumors  and 
muscle  metastases  (Weigert)  were  found.     In  one  case  Pel  found  leucocytosis 


o   *- 

bD  ^> 

o.S 
"35 'a 
o  « 

O 
§| 

tn  •« 

«*- 

ta  o 

IB 

a. a 

o    „ 


§5 
•BM 

'c3  xi 


"-1   to 

>  o 
u 

3 


442  THE  MYOPATHIES 

intra  vitam  and  leucocytic  accumulations  in  the  parenchymatous  organs. 
Taking  all  in  all,  however,  the  anatomic  findings  thus  far  do  not  explain  fully  the 
severe  functional  disturbances  of  myasthenia.  Whether  the  morbid  preponder- 
ance of  the  "light  muscular  fibres,"  recently  found  by  Knoblauch,  at  the 
expense  of  the  "red  dark  ones,"  in  myasthenia  and  the  comparative  biolog- 
ical reasons  of  the  author  for  the  pathogenesis  of  myasthenia,  will  acquire 
importance,  is  as  yet  by  no  means  sure. 

Etiology. — The  real  cause  is  unknown.  Acute  infectious  diseases, 
however  (influenza,  traumata,  intoxications,  over-exertions,  etc.),  are 
credited  as  excitant  factors.  The  latter  factor  seems  especially  plausible 
in  an  unpublished  case  of  Erb,  that  of  an  old  Boer,  who  was  chased  on 
horseback  with  Dewet  by  the  English  for  weeks  with  minimal  resting 
periods. 

Often,  however,  there  is  no  obvious  causal  factor  to  be  found. 
The  assumption  that  a  secretory  disturbance  of  the  glandulse  parathy- 
reoideae  is  the  cause  of  myasthenia  (Lundborg,  Chvostek)  has  not  yet  been 
proven  in  any  way. 

Of  great  importance,  however,  is  the  proof  first  adduced  by  Oppenheim, 
that  many  myasthenics  exhibit  a  striking  congenital  inhibition  in  development 
and  deformities  (anomalies  in  the  nervous  apparatus,  polydactylia  in  feet  and 
hands  — Oppenheim — total  aplasia  of  the  genital  organs  and  hyperplasia  of 
the  lobes  of  the  lungs — observation  of  the  author) .  These  findings  stamp  the 
patient  doubtless  as  belonging  to  the  congenital  hypoplasts,  in  regard  to 
which  Edinger's  theory  of  abnormal  "using  up"  and  insufficient  reparation 
in  the  nervous  system,  must  seem  very  probable  to  us,  and  explicative  of 
many  things. 

The  complications,  by  which  the  disease  is  rarely  accompanied — 
hysteria,  Basedow's  disease,  dystrophia  musculorum,  Banti's  disease 
(Mohr) ,  can  be  of  little  assistance  in  the  aetiological  research. 

The  differential  diagnosis  may  be  difficult  in  the  beginning,  especially 
relative  to  hysteria,  but  is  cleared  up  by  the  type  of  the  paralysis  of  the  eye 
muscles,  the  disturbance  in  deglutition,  the  strictly  remittent  character  of 
the  paralyses  and  finally  the  My  a.  R.  Against  the  diagnosis  of  atrophic 
and  pseudo-bulbar  paralysis  we  are  protected,  on  the  one  hand,  by  observa- 
tion of  the  degenerative  atrophies,  on  the  other  by  the  disturbances  of  the 
lateral  pyramidal  tracts  (spasms,  increased  reflexes)  in  those  cases.  Differ- 
entiation from  a  polyneuritic  bulbar  paralysis  is  more  difficult  in  the  begin- 
ning; the  same  difficulty  is  met  with  in  cerebral  syphilis,  Landry's  paralysis 
and  especially  poliencephalomyelitis  {Oppenheim).  The  absence  of  the 
quantitative  and  qualitative  electrical  changes  and  the  appearance  of  the 
Mya.  R.  will,  however,  decide  the  differential  diagnosis,  even  in  these  cases. 
The  prognosis  is  usually  unfavorable.  Although  Erb,  Goldflam,  H. 
Steinert  and  others  observed  recoveries  in  light  cases,  most  cases  after  shorter, 


MYOTONIA  CONGENITA  443 

or,  as  usually  happens,  longer  duration,  end,  as  has  been  described  above, 
fatally. 

Treatment  can  consist  only  of  great  conservation  of  effort,  rest  and  suit- 
able nourishment;  besides  tonics  may  be  useful.  All  severe  electric  or 
active  and  passive  gymnastic  procedures,  which  could  only  increase  the 
exaggerated  tendency  of  the  muscles  to  become  fatigued,  are  contra-indicated. 
Treatment  by  baths  is  to  be  employed  only  with  attentive  control  of  respira- 
tion and  pulse;  better,  not  at  all  (Steinert).  The  stomach-tube,  which  is 
occasionally  unavoidable,  when  the  swallowing  apparatus  is  totally  paralyzed, 
is  to  be  used  only  with  the  greatest  caution,  since  cases  of  death  during  its 
passage  have  been  observed  (Oppenheim). 

4.  MYOTONIA  CONGENITA  (Oppenheim) 

This  disease,  first  described  by  Oppenheim  in  1894,  since  then  described 
in  only  about  ten  cases,  seems  to  be  nearly  always  present  at  birth,  though 
occasionally — in  milder  degrees — it  was  noticed  first  in  the  2nd  or  3rd  year 
of  life.  The  children  thus  affected  are  conspicuous  in  that  they  cannot  move 
their  limbs,  especially  the  lower  extremities,  spontaneously:  the  limbs  are 
completely  paralyzed  and  flaccid,  the  muscular  tonus  is  markedly  reduced, 
the  limbs  hang  loosely  from  the  joints.  In  the  arms,  the  disturbance  is 
usually  slighter  and  here  the  weakness  of  the  movements  is  more  prominent. 
The  motility  of  the  trunk  and  neck,  on  the  other  hand,  is  scarcely  ever  dis- 
turbed. Involvement  of  the  muscles  of  the  head,  face,  tongue,  eyes,  larynx 
and  diaphragm  has  not  yet  been  observed.  The  sensory  cerebral  nerves, 
the  sensory  functions,  the  sphincters  and  the  intellectual  development,  also, 
seem  not  to  suffer  in  the  disease. 

The  muscles,  though  they  feel  limp  and  withered,  show  no  real  atrophies 
and  no  fibrillary  twitchings.  The  direct  and  indirect  electrical  excitability 
in  the  paralyzed  as  well  as  in  the  apparently  sound  muscles  is  usually 
reduced,  quantitatively  to  a  large  degree,  frequently  even  to  the  point  of 
entire  disappearance  of  the  excitability,  and  a  suggestion  of  the  reaction  of 
degeneration  has  been  observed. 

The  tendon  reflexes  are  nearly  always  abolished,  not  only  in  the  extrem- 
ities affected,  but  also  in  those  that  are  normally  movable;  the  cutaneous 
reflexes  are  mostly  described  as  normal. 

Anatomic  findings  are  known  so  far,  only  in  one  case  (Spiller):  the 
entire  nervous  system  proved  to  be  intact;  the  muscles,  on  the  other  hand, 
showed  hyaloid  changes,  looked  cloudy  and  had  only  indistinct  striation; 
the  individual  fibrils  were  abnormally  small,  the  fat  containing  connective 
tissue  increased.  Signs  of  inflammation  and  breaking  down  were  lacking. 
Other  authors  corroborated  these  findings  in  sections  of  muscle  taken  from 
the  living  body  (Reyher  and  Helmholtz),  whereas  Bing  found  normal  structure. 


444  THE  MYOPATHIES 

Oppenheim  sees  the  very  essence  of  myatonia  in  an  inborn  retarded 
development  of  the  musculature;  the  anatomical  findings  for  the  most  part, 
seem  to  speak  for  this.  Bernhardt  is  rather  inclined  to  assume  an  injury  to 
the  peripheral  nerves,  somewhat  like  a  generalized  polyneuritis  of  autotoxic 
or  infectious  origin  (partly  on  the  basis  of  cases,  which  were  notcongenitally 
diseased).  At  any  rate,  the  pathogenesis  of  the  disease  is  greatly  in  need 
of  classification  by  further  clinical  and  anatomical  findings,  which  first  and 
foremost  must  prove  if  there  is  really  such  a  disease  entity. 

The  differential  diagnosis  must  consider  especially,  poliomyelitis 
anterior  and  the  peripherally  and  spinally  induced  paralyses  intra-partum. 
The  absence  of  atrophies  and  disturbances  in  sensation,  the  generalized, 
almost  always  symmetrical  affection  of  the  muscles  and  the  change  in 
electrical  excitability,  which  mostly  affects  all  the  muscles,  distinguishes, 
however,  myatonia  from  those  diseases.  From  a — probably  exceedingly 
rare — general  polyneuritis  of  nursing  infants,  the  disease,  it  seems  to  me, 
can  not  be  distinguished.  Finally,  I  desire  to  earnestly  point  out,  that 
nervously  normal,  but  somewhat  atrophic  or  rachitic  children  may  show  a 
high  degree  of  hypotonia  of  the  muscles  and  joints  (which,  to  a  certain 
degree,  is  physiologically  peculiar  to  early  childhood).  This  likewise 
furnishes  a  differential  diagnostic  source  of  error. 

The  course  is  always  extremely  slow.  The  prognosis,  however,  is  not 
bad,  since  in  some  of  the  cases,  in  the  course  of  time,  a  more  or  less  complete 
disappearance  of  the  disturbances  has  been  observed. 

Treatment  must  consist  in  the  use  of  general  tonic  means,  of  careful 
strychnine  medication,  massage  and  the  electric  current;  possibly  ortho- 
pedic procedures  may  also  become  necessary. 


V 
DISEASES  OF  THE  BRAIN 

NORMAL  AND  PATHOLOGICAL  PHYSIOLOGY  OF  1HE  BRAIN  x 

BY 

HUGO  LIEPMANN  (Berlin) 

I.   INTRODUCTORY  REMARKS  ON  THE  ANATOMY,  PHYSIOLOGY, 
AND  PATHOLOGY  OF  THE  BRAIN 

i.  The  Membranes  of  the  Brain 

The  brain,  covered  by  two  membranes,  the  dura  mater  and  pia-arach- 
noid,  lies  enclosed  within  the  cranium. 

The  topographical  relations  of  the  different  parts  of  the  brain  to  the 
skull  are  shown  in  Fig.  109. 

The  dura  mater  is  the  periosteum  of  the  bones  of  the  skull.  In  the 
healthy  adult  it  is  attached  firmly  only  to  the  base  of  the  skull,  and  can  be 
easily  detached  from  the  convexity.  Extensions  of  it  project  sagitally 
into  the  spaces  between  the  hemispheres  of  the  cerebrum  (proc.  falcif. 
major.),  the  hemispheres  of  the  cerebellum  (proc.  falcif.  minor.),  and  trans- 
versely between  the  cerebellum  and  the  base  of  the  occipital  lobe  (tentorium). 

It  forms,  in  places,  by  splitting  into  two  layers,  the  so-called  sinuses. 
They  contain  blood.  Its  fibrous  layer  forms  sheaths  for  the  cranial  nerves 
as  they  pass  through  the  foramina. 

Its  nervous  supply  arises  partly  from  the  sympathetic  and  partly  from 
the  trigeminal. 

It  is  impossible  to  give  the  varying  views  of  the  leading  specialists  on 
the  many  mooted  points. 

The  names  of  authors  are  purposely  not  quoted,  except  in  a  few  cases, 
in  which  a  fundamental  theory  is  connected  with  a  name.  The  soft 
membrane  is  divisible  into  two,  an  outer  layer,  the  arachnoid,  and  an  inner, 
the  pia  mater. 

The  pia  lines  all  fissures,  following  closely  the  surface  of  the  brain  and 
enters  the  ventricles  as  the  tela  chorioidea,  while  the  arachnoid  membrane 
is  stretched  across  the  fissures.  The  two  are  closely  connected  by  numerous 
trabecular  of  connective  tissue,  so  that  pathologically,  they  may  be  treated 

1  The  need  of  brevity  prevents  minute  discussion  and  compels  us  to  present  a  very  intricate 
matter  in  as  simple  a  form  as  possible. 

445 


446 


DISEASES  OF  THE  BRAIN 


as  one.  Between  the  dura  and  the  pia  arachnoid  lies  the  subdural  space, 
which  contains  scarcely  a  trace  of  fluid.  Even  in  disease  it  is  not  a  place 
for  the  collection  of  fluids. 

On  the  other  hand  in  the  subarachnoid  space,  between  the  arachnoid 
membrane  and  the  pia,  because  of  its  communication  with  the  ventricles,  is 
found  normally  some  cerebro-spinal  fluid,  which,  in  progressive  paralysis, 
senile  atrophy,  etc.,  is  often  much  increased  (hydrocephalus  externus). 


Fig.   109. — Relation   between    the    cranium    and    the'  cerebral    surface.     (After    Bardeleben- 

Haeckel.) 


2.  Circulation  in  the  Brain 

The  brain  receives  blood  from 

1.  Both  vertebral  arteries. 

2.  Both  internal  carotid  arteries. 

Both  vertebral  arteries  unite  in  passing  from  the  medulla  oblongata  to 
the  pons  and  form  the  basilar  artery,  which  before  reaching  the  pons  divides 
into  the  two  posterior  cerebral  arteries  coursing  backward  and  laterally. 
— Art.  cerebr.  post. 

The  internal  carotid  branches  as  follows  on  both  sides: 

(a)  Anterior  cerebral  artery  (Art.  cerebr.  ant.) . 
These  two  are  connected  by  the  anterior  communicating  artery. 


CEREBRAL  CIRCULATION 

(b)  The  middle  cerebral  artery  (Art.  foss.  Sylvii). 

(c)  The  posterior  communicating  artery  (Art.  commun.  post.). 

(d)  The  chorioidal  artery  (Art.  chorioidea). 


447 


p  ,'OpRL. 


Gyv  temp- prof 


Fig.  i  10. — Areas  of  the  convexity  as  supplied  by  the  cerebral  arteries.  White,  area  of  the 
art.  fossae  Sylvii.  Red,  of  the  art.  cerebri  anterior.  Blue,  of  the  art.  cerebri  posterior.  The 
other  abbreviations  as  in  Fig.  112. 


Fig.  hi. — Areas  of  the  median  surface  as  supplied  by  the  cerebral  arteries. 
Colors  as  in  Fig.  no. 

In  the  posterior  communicating  artery  is  mixed  the  blood  from  the  carotid 
with  that  from  the  vertebral,  as  it  joins  the  posterior  cerebral  artery.    'By 


448 


DISEASES  OF  THE  BRAIN 


means  of  the  anterior  and  posterior  communicating  arteries  the  circle  of 
Willis  is  completed.     Variations  in  it  are  frequent. 
The  three  main  cerebral  arteries  are  therefore: 

i.  The  anterior  cerebral  artery.  ]  —     .   ,         -_■  -  .,  N 

„,,         . ,  „  ,  t  (Both  from  the  carotid.) 

2.  The  middle  cerebral  artery.     J 

3.  The  posterior  cerebral  artery  (from  the  basilar  artery). 

These  main  arteries  send  short  branches  into  the  inner  part  of  the  brain 
(central  arteries),  especially  to  the  ganglia,  and  long  branches  to  the  cortex, 
and  the  adjacent  white  matter  (cortical  arteries). 

The  short  and  long  (cortical)  branches  form,  in  themselves,  two  complete 
systems  of  circulation,  not  interdependent  but  independent  of  each  other, 
as  there  are  no  anastomoses  between  them. 


F.tr: 


P  /  Op.RL 

1. ope re.        v- 


Gyr  temp.  prof. 

Fig.  112. — Convolutions  and  fissures  of  the  convexity.  (Using  a  design  of  Flechsig.)  S.h., 
S.a.,  S.f,  Ramus  horizontalis,  ramus  ascendens  or  verticalis  and  ramus  posterior  of  trie  Sylvian 
fissure;  Of.R.,  operculum  Rolandi;  f.tr.,  pars  triangularis  of  the  third  frontal  convolution; 
P.  of  ere,  pars  opercularis  of  the  third  frontal  convolution;  P.O.,  pars  orbitalis  of  the  third  frontal 
convolution.  The  designation  Gyr.  suframargin  ought  to  begin  more  in  the  front  immediately 
behind  the  Gyr.  centr.  fost. 

The  short  branches  (central  arteries)  have  among  themselves  as  well  as 
with  other  arteries  few  anastomoses,  so  that  they  are  designated  terminal 
arteries. 

The  long  branches  course  along  on  the  surface  of  the  brain,  supply  the 
cortex  and  adjacent  white  matter,  and  have,  among  themselves,  anastomoses. 

Of  the  central  branches,  those  that  supply  the  inner  capsule,  the  lenticular 
nucleus  and  the  optic  thalamus,  are  of  especial  importance.  (Lenticulo- 
striate  and  lenticulo-optic  arteries).  The  region  supplied  by  these  non- 
anastomosing  vessels,  is  the  place  of  preference,  for  the  bursting  of  blood- 
vessels and  cerebral  softening;. 


CEREBRAL  CIRCULATION 


449 


The  anterior  cerebral  artery  (main  branch:  the  corpus  callosum  artery: 
art.  corp.  callosi)  supplies  both  superior  frontal  convolutions,  the  base  of  the 
frontal  lobes  and  the  medial  parts  of  the  central  hemisphere  (gyrus  f  ornicatus, 
the  paracentral  lobule  including  the  precuneus,  and  the  corpus  callosum). 

Since  the  paracentral  lobule  is  the  seat  of  the  leg-center,  an  obstruction  in 
this  branch  of  the  anterior  cerebral  artery  produces  an  isolated  paralysis  of 
the  leg. 

Disease  of  the  middle  cerebral  artery  or  the  artery  of  the  fissure  of  Sylvius 
has  especial  importance  in  disturbances  of  speech.  Its  short  central  branches 
have  already  been  mentioned,  as  the  important  lenticulo-striate,  etc. 


Fig.   113. — Convolutions  and  fissures  of  the  medial  surface.     (Using  a  design  of  Flechsig.)  Sulc. 
calhsom.,  Sulcus  callosomarginalis;  Lobus  parac.,  lobus  paracentralis. 

Its  5  to  7  main  cortical  branches  supply  the  third  frontal  convolution 
the  island  of  Reil,  the  central  convolutions,  the  parietal  lobes,  and  the 
superior  convolutions  of  both  temporal  lobes. 

The  posterior  cerebral  artery  supplies  the  walls  of  the  ventricles.  Its 
short  branches  go  to  the  optic  thalamus,  the  cms  cerebri,  the  geniculate 
body,  the  chorioid  tela,  etc.  By  short  branches  it  supplies  also  the  splenium 
of  the  corpus  callosum. 

It  divides  into  two  main  cortical  branches : 

1.  The  temporal  artery  which  supplies  the  undersurface  of  the  temporal 
lobe. 

2.  The  occipital  artery  which  supplies  the  occipital  lobe. 

Of  the  branches  of  the  latter  the  calcarine  artery  is  especially  important, 
because  it  supplies  the  vicinity  of  the  calcarine  fissure — that  is  the  visual 
center — and  the  optic  radiation. 
29 


450  DISEASES  OF  THE  BRAIN 

The  cerebellum  and  the  pons  are  supplied  by  branches  of  the  basilar  artery 
(the  cerebellar  artery). 

,The  different  parts  of  the  brain  are,  therefore,  supplied  as  follows: 

The  cortex  of  the  cerebrum  and  its  white  matter;  by  the  cortical  branches  of 
the  three  main  arteries,  thus: 

The  frontal  brain:  basal,  medial  and  upper  convexity,  by  the  anterior 
cerebral  artery;  the  rest  of  its  convexity,  especially  F.  m.  and  F.  i.  by  the 
middle  cerebral  artery. 

The  central  convolutions,  the  island  of  Rett  and  the  convexity  of  the  parietal 
lobes  by  the  middle  cerebral  artery. 

The  medial  parietal  lobes  (paracentral  lobule  and  prsecuneus)  gyrus 
fornicatus,  by  the  anterior  cerebral  artery.  (Prsecuneus  partly  also  by  the 
posterior  cerebral  artery.) 

The  temporal  lobes,  the  two  upper  convolutions  by  the  middle  cerebral 
artery;  the  rest,  especially  the  base,  by  the  posterior  cerebral  artery. 

The  occipital  lobes,  by  the  posterior  cerebral  arteries. 

The  large  ganglia  and  inner  capsule,  by  the  central  branches  of  the  three 
main  arteries. 

The  corpus  callosum  by  the  anterior  cerebral  artery;  the  splenium  by  the 
posterior  cerebral  artery. 

The  middle  basal  region,  by  the  posterior  communicating  artery. 

The  cerebral  peduncles,  by  the  posterior  cerebral  and  the  posterior  com- 
municating arteries. 

The  quadrigeminal  bodies,  by  the  posterior  cerebral  artery. 

The  pons,  cerebellum  and  medulla  oblongata,  by  the  superior  cerebellar 
artery,  the  basilar  artery  and  the  inferior  cerebellar  artery,  the  vertebral 
artery. 

Veins  of  the  Brain. 

The  veins  of  the  brain  have  no  valves  and  are  connected  by  innumerable 
anastomoses.  They  drain  into  the  dural  sinuses  (sinus  longitudinalis,  etc.) , 
which  finally  empty  their  blood  into  the  internal  jugular  vein.  The  veins 
from  the  corpus  striatum  and  the  choroid  plexuses  of  the  ventricles,  unite  in 
the  azygos  great  cerebral  vein  {vena  magna  Galeni)  below  the  corpus  cal- 
losum.    This  empties  its  blood  into  the  straight  sinus  (sinus  rectus). 

3.  Morphology  of  the  Brain 

The  weight  of  the  brain  in  man  averages  about  49  ounces,  in  woman 

4  ounces  less. 

On    the    grounds    of    embryological    development    one    distinguishes: 
1.  The  end  brain  (telencephalon);  both  hemispheres  with  the  anterior 

ganglia;  caudate  nucleus,  lenticular  nucleus,  amygdaloid  nucleus  and  claus- 

trum.     Its  cavity:  the  lateral  ventricles. 


MORPHOLOGY  OF  THE  BRAIN  451 

2.  The  interbrain  (diencephalon) :  the  optic  thalamus  with  its  appendages, 
especially  the  important  geniculate  body  and,  at  the  base,  the  chiasma  and 
optic  tract.     Its  cavity:  the  third  ventricle. 

These  together  constitute  the  forebrain  (prosencephalon). 

3.  The  midbrain  (mesencephalon) :  the  quadrigeminal  bodies,  inferior 
lemniscus,  red  nucleus  and  the  nuclei  of  the  third  and  fourth  nerves,  cerebral 
peduncles  (tegmentum  and  base)  with  the  substantia  nigra.  Of  the  cerebral 
cavities  the  aqueduct  of  Sylvius  corresponds  to  the  midbrain. 

4.  The  hind-brain  (metencephalon  or  rhombencephalon) :  the  pons  and 
cerebellum. 

5.  The  after-brain  (myelencephalon) :  the  medulla  oblongata. 

The  cavity  of  the  hind  and  after  brain  is  the  fourth  ventricle.  The  end 
towards  the  spinal  cord  forms  the  lowest  part  of  the  pyramidal  crossing. 

The  forebrain,  together  with  the  midbrain  is  frequently  spoken  of  as 
the  greater  brain  (cerebrum)  as  against  the  cerebellum,  the  medulla  and  the 
pons. 

The  "brain  stem"  is  the  name  for  the  medulla,  pons,  midbrain,  inter- 
brain and  the  cerebral  ganglia  in  contrast  to  the  cortex  and  the  white  matter 
of  the  hemispheres. 

Each  hemisphere  is  divided  into  frontal,  parietal,  temporal  and  occipital 
lobes. 

The  largest  fissure,  the  fissure  of  Sylvius,  separates  the  temporal  lobe 
from  the  frontal  and  parietal  lobes.  Many  claim  that  the  frontal  lobe  is 
separated  from  the  parietal  lobe  by  the  fissure  of  Rolando  or  central  fissure, 
so  that  the  anterior  central  convolution  is  counted  as  belonging  to  the  frontal, 
the  posterior  central  convolution  as  belonging  to  the  parietal  lobe.  But  both 
central  convolutions  are  often  spoken  of  as  a  whole:  the  central  region  or 
region  of  Rolando,  and  in  that  case  it  counts  as  an  independent  part  lying 
between  the  frontal  and  parietal  lobes. 

Between  the  frontal,  parietal,  and  temporal  lobes,  in  the  depths  of  the 
fissure  of  Sylvius  lies  the  island  of  Reil  (insula).  The  parts  of  the  frontal 
brain  and  of  the  central  convolutions  covering  it  are,  hence,  called  the  oper- 
culum, and  that  part  of  the  operculum  that  belongs  to  the  central  convolutions, 
the  Rolandic  operculum,  that,  which  belongs  to  the  inferior  frontal  cortex, 
the  frontal  operculum  (Broca's  area). 

The  occipital  lobe  is  divided  sharply  from  the  parietal  lobe,  only  on  the 
medial  surface,  by  the  parieto-occipital  fissure;  a  distinct  boundary  is  lacking 
at  its  convexity. 

(a)  Convolutions  and  Fissures  of  the  Convexity. 

The  surface  of  the  frontal  lobe  is  divided  by  two  fissures  into  three  con- 
volutions, the  superior  or  first,  F  s,  the  middle  or  second,  F  m,  and  the  inferior 
or  third,  F  i,  frontal  convolution. 


452  DISEASES  OF  THE  BRAIN 

The  inferior  frontal  convolution  is  divided  by  the  anterior  (horizontal) 
and  the  ascending  (vertical)  branch,  (ramus  ascendens)  of  the  fissure  of 
Sylvius  into  three  parts  (Fig.  no): 

i.  In  front,  pars  orbitalis, 

2.  Between  the  horizontal  and  the  ascending  branch,  the  pars  triangularis 
and 

3.  Behind  the  ascending  branch,  the  before  mentioned  frontal  operculum 
which  posteriorly  gradually  disappears  in  the  Rolandic  operculum. 

The  parietal  lobe  extends  anteriorly,  if  we  consider  both  central  con- 
volutions as  the  Rolandic  region  per  se,  up  to  the  fissure  that  marks  the  back 
boundary  of  the  posterior  central  convolution  and  is  divided  by  the  inter- 
parietal fissure  into  a  superior  and  an  inferior  lobule. 

The  inferior  parietal  lobule  is  divided  into 

1.  The  anterior  part  or  the  supramarginal  gyrus  which  surrounds  the 
posterior  end  of  the  fissure  of  Sylvius  and,  below,  forms  the  extension  of  the 
superior  temporal  convolution  posteriorly,  and  which  extends  anteriorly  to 
the  posterior  central  'convolution  (the  French  designate  only  that  part  of 
the  convolution  that  touches  the  posterior  end  of  the  fissure  of  Sylvius,  as  the 
gyrus  supramarginalis) . 

2.  The  posterior  part,  or  the  angular  gyrus  (pli  courbe)  which  surrounds 
the  posterior  end  of  the  temporal  fissure.  Between  the  supramarginal  and 
angular  gyri,  the  inconstant  fissure  of  Jensen. 

This  angular  gyrus,  often  interrupted  by  a  fissure,  continues  into  the 
occipital  lobe. 

To  simplify  orientation  one  must  remember  that  the  supramarginal 
gyrus  is  placed  at  the  posterior  end  of  the  fissure  of  Sylvius,  and  the  angular 
gyrus  at  the  posterior  end  of  the  superior  temporal  fissure. 

The  convexity  of  the  temporal  lobe  is  divided,  by  the  above-mentioned 
superior  temporal  fissure  (sulcus  temp,  sup.)  and  the  middle  temporal  fissure, 
into  three  convolutions,  the  superior,  middle,  and  inferior  temporal  convolu- 
tions. The  superior  temporal  convolution  continues  posteriorly  into  the 
parietal  lobe,  especially  the  supramarginal  gyrus  corresponding  to  the  end  of 
the  fissure  of  Sylvius,  the  middle  temporal  convolution,  especially  into  the 
angular  gyrus  and  the  inferior,  into  the  corresponding  occipital  convolutions. 

Coursing  along  the  surface  of  that  part  of  the  temporal  lobe,  that  is 
turned  toward  the  island  of  Reil — concealed  in  the  fissure  of  Sylvius — taking 
their  origin  in  the  surface  of  the  superior  temporal  convolution,  1  or  2  cross 
convolutions  (gyri  temporalis  profundi  or  transversi,  also  called  the  convolutions 
of  Heschl),  pass  backwards  and  inwards  to  the  parietal  region  behind  the 
island  of  Reil. 

The  occipital  lobe  has  anteriorly  no  sharp  boundary.  As  an  artificial 
boundary  one  assumes  a  line  that  unites  the  upper  end  of  the  parieto- 
occipital fissure  with  the  incisura  praeoccipitalis. 


MORPHOLOGY  OF  THE  BRAIN  453 

(b)  The  Median  Surface  and  Base  of  the  Brain 

The  median  surface  of  the  hemispheres  shows  first  of  all  the  entrance  of 
the  corpus  callosum,  that  great  commissural  system,  which  connects  both  the 
symmetrical  and  the  asymmetrical  parts  of  the  two  hemispheres. 

Below  the  corpus  callosum,  and  bent  from  before  backwards  in  a  similar 
curve,  we  see  the  fornix  (the  thickened  ends  of  the  hemispheres),  which 
continues  towards  the  back  in  the  fimbria  of  the  inferior  horn  of  the  lateral 
ventricle  (Fig.  113). 

Parallel  to,  and  above  the  corpus  callosum,  lies  one  of  the  main  furrows 
of  the  median  surface,  the  sulcus  cinguli  or  callosomarginal  sulcus,  which 
turns  posteriorly  upwards  to  the  edge  of  the  hemisphere  and  ends  in  a  small 
notch  behind  the  end  of  the  posterior  central  convolution. 

The  concentric  convolution  between  this  furrow  and  the  corpus  callosum, 
is  called  the  gyrus  fornicatus.  What  lies  in  front  of,  and  above  it,  is  classed 
with  the  frontal  brain  (gyrus  rectus  and  medial  part  of  the  superior  frontal 
convolution);  only  the  hindmost  part  of  this  concentric  convolution,  which 
corresponds  to  the  central  convolutions  of  the  convex  surface,  is  considered 
as  a  small  lobe,  per  se,  called  the  paracentral  lobule  (lobus  par acentr alls). 

The  posterior  part  of  the  gyrus  fornicatus  (back  of  the  ascending  branch 
of  the  sulcus  cinguli) ,  spreads  into  the  precuneus,  which  is  part  of  the  parietal 
lobe.  Its  posterior  boundary  is  the  second  main  fissure  of  the  median  sur- 
face: the  parieto-occipital  fissure,  mentioned  before  as  the  boundary  between 
the  parietal  and  the  occipital  lobes.  Into  this  fissure,  anteriorly,  passes  a 
third  very  important  main  fissure,  the  calcarine  fissure  (fissura  calcarina),  run- 
ning sagitally  postero-anteriorly. 

The  wedge-shaped  part  of  the  convolution,  lying  between  the  fissures, 
is  the  cuneus. 

Under  the  calcarine  fissure  lies  the  gyrus  lingualis,  and  under  this,  sepa- 
rated from  it  by  the  occipito-temporal  sulcus  (collateralis)  the  gyrus fusiformis, 
which  continues  anteriorly  through  the  temporal  lobe.  Both,  the  gyrus 
lingualis  and  fusiformis  belong  to  the  basal  surface.  The  gyrus  fornicatus 
continues  backwards  and  upwards  into  the  precuneus.  Towards  the  lower 
and  back  part,  it  winds  about  the  splenium  of  the  corpus  callosum,  and  here 
continues  into  the  gyrus  hippocampi,  which  extends  to  the  anterior  end  of  the 
median  temporal  lobe.  The  hook-like  bend,  which  the  concentric  convolu- 
tions (gyrus  fornicatus  and  gyrus  hippocampi)  show  at  their  anterior  extrem- 
ity, is  called  the  uncus. 

In  the  gyrus  hippocampus,  the  gyrus  lingualis  continues  from  the  back, 
so  that  in  the  gyrus  hippocampus  the  gyrus  fornicatus  and  the  gyrus  lingualis 
are  united. 

As  is  evident,  the  median  surface,  posteriorly,  gradually  passes  into  the 
basal  surface. 


454 


DISEASES  OF  THE  BRAIN 


On  the  basal  surface,  between  the  temporal  and  the  occipital  lobes,  no 
sharp  line  can  be  drawn.  The  inferior  temporal  convolution  helps  to  form 
the  base. 

Medially  from  it,  separated  by  the  inferior  temporal  fissure,  lies  the 
before  mentioned  fusiform  gyrus,  and  medially  in  front  of  this,  lies  the 
hippocampal  gyrus,  in  back  of  it  the  lingual  gyrus.  The  fusiform  gyrus  is 
separated  from  them  by  the  occipito-temporal  fissure   (collateral  fissure) 


Cornmis. 
ant. 


Fig.  114. — Lateral  view  of  inter-  and  midbrain.     The  connections  between  corp.  geniculat. 
laterale  {Corp.  g.  lat.)  and  the  occipital  lobe,  not  indicated.     {After  Edinger.) 

which  extends  through  the  entire  undersurface,  almost  to  the  temporal  pole. 
In  front  the  base  shows  between  the  central  peduncles,  the  mammillary 
bodies,  the  pituitary  body,  the  optic  tracts,  the  anterior  and  posterior 
perforated  spaces. 

At  the  base  of  the  frontal  lobe  the  gyrus  rectus,  i.  e.,  the  basal  continuation 
of  the  superior  frontal  convolution  is  cut  off  towards  the  inner  side,  by  the 
olfactory  sulcus  in  which  the  olfactory  lobe  is  situated.  Outwards  from  it, 
lies  the  basal  part  of  the  middle  frontal  convolution,  and  separated  from  it 
by  the  sulcus  orbitalis,  the  basal  (also  called  orbital)  part  of  the  inferior 


HISTOLOGICAL  STRUCTURE  OF  THE  CORTEX 


455 


frontal  convolution  (pars  orbitalis  of  F.  i.) .  To  understand  the  form  relations 
of  the  inter-,  mid-,  after-,  and  hind-brain,  one  needs  only  to  look  at  Figs. 
114  and  115. 


VentrLculus  tertius 


Interbrain 


Midbrain 


Hind-brain 


After-brain 


Peduncle 


Corp.  restif. 
Aia  cine  re  a 


Cerebelum 


Spinal  cord 
Fig.  115. — View  of  inter-  mid-  and  after-brain.     {After  Edinger.) 


4.  Histological  Structure  of  the  Cortex 

The  cellular  cortical  structure,  as  proven  by  embryology  and  comparative 
anatomy,  may  be  divided  into  six  layers: 

1.  Lamina  zonalis  (molecular  layer). 

2.  Lamina  granulans  externa  (outer  granular  layer). 

3.  Lamina  pyramidalis  (pyramid  layer). 

4.  Lamina  granulans  interna  (inner  granular  layer). 


456 


DISEASES  OF  THE  BRAIN 


5.  Lamina  ganglionaris  (ganglia  cells  layer). 

6.  Lamina  multiformis  (polymorphous  or  spindle  celled  layer). 

This  six-layered  type,  established  on  embryologic  grounds,  in  the  whole 
cortex  of  the  cerebrum,  is,  besides,  in  Fig.  116  divided  into  subdivisions 
(III  a  and  b,  VI  a  and  b),  and  is  subject  to  numerous,  local  modifications 
which  frequently  are  sharply  limited;  they  consist  in  increase,  diminution,  or 


Fig.  116. — Cyto-  and  myelo-architectonic  structure  of  the  cortex.  The  Arabic  figures  at  the 
right  designate  the  myelo-architectonic  layers,  xo,  Pars  afibrosa  laminae  tangentalis;  ia,  pars 
externa  laminae  tangentalis;  ib,  pars  intermedia  laminae  tangentalis;  ic,  pars  interna  lamina?, 
tangentalis;  2,  lamina  dysfibrosa;  ^aa,  stria  Kaesi-Bechterewi;  3a/?,  regio  typica  partis  superficialis 
laminae  suprastriatae;  36,  pars  profunda  laminae  suprastriatae;  4,  stria  Baillargeri  externa;  5a, 
lamina  intrastriata;  $b,  stria  Baillargeri  interna;  6a(3,  lamina  substriata;  6a/3,  lamina  limitans 
externa;  66a,  lamina  limitans  interna;  6fy3  substantia  alba.  The  Roman  figures  at  the  left 
designate  the  cyto-architectonic  layers.     I  to  VI  see  text.     (After  Vogt  and  Brodmann.) 

transposition  of  single  layers,  and  the  inner  granular  layer  may  disappear, 
or  by  fission,  double,  and  other  cellular  formations,  i.  e.,  giant  pyramid  cells 
may  appear  and  finally  variations  in  the  thickness  and  size  of  the  cells,  in 
their  density,  and  the  breadth  of  the  layers,  are  found.  Accordingly,  in  the 
entire  cortex  about  50  such  modifications  of  the  layers  can  be  distinguished,  and 
corresponding  regions  marked  off.  In  general,  their  boundaries  do  not 
correspond  to  the  fissures  and  convolutions  (Fig.  117). 

Of  special  importance  are  two  extreme  variations  of  cell  layers : 
1.  The  giant  pyramidal  or  motor  type  in  the  anterior  central  convolution 
(Fig.  118).     The  fourth  layer  is  wanting. 


HISTOLOGICAL  STRUCTURE  OF  THE  CORTEX 


457 


2.  The  calcarine  type  with  duplication  of  the  inner  granular  layer  in  the 
immediate  vicinity  of  the  calcarine  fissure  (corresponding  to  the  clinico- 
pathologically  defined  visual  sphere  of  Henschen) . 

The  regions  as  mapped  off  according  to  the  cortical  cell  architecture 
(cyto-architectonically),  correspond  only  partly,  and  only  approximately  to 
the  regions  mapped  out  by  Flechsig,  on  the  basis  of  myelin-development 
(myelogenesis) . 

Beside  the  cellular  layer,  differences  in  density,  arrangement,  and  caliber 
of  the  medullated  nerve  fibres  of  the  cortex,  condition  a  different  cross-cut 


Fig.  117. — The  cyto-architectonic  cortical  regions  of  the  convexity.     (After  Brodman.) 

division  of  various  places  of  the  cortex,  which  gives  boundaries  of  about  the 
same  cortical  fields,  as  cyto-architectonics  (Fig.  116). 

The  relation  of  the  horizontal  fibrillation  of  the  cortex,  to  the  radial 
medullated  nerve  fibres,  if  we  overlook  local  peculiarities,  presents  the 
following  appearance: 

1.  Tangential  layer. 

2.  Supraradial  felt  work  (Edinger). 

3.  Intraradial  felt  work,  containing  the  internal  and  external  lines  of 
Baillarger,  which  form  only  a  density  in  the  fibres  that  runs  horizontally 
through  the  cortex. 

More  detailed  investigation  has  led  to  a  further  separation  into  divisions 


458 


DISEASES  OF  THE  BRAIN 


**    *    •     »  ■•      4'-    •  '•     J.  ■' 


,  » 


and  subdivisions,  which  we,  in  the  above  scheme 
of  O.  Vogt  (Fig.  1 1 6)  have  brought  into  relation 
with  the  cyto-architectonic  fundamental  type  of 
Brodmann. 

The  peculiarly  strong  marking  of  this  hori- 
zontal fibre  layer  in  the  calcarine  region  is  known 
as  the  Vicq  d'Azyr  stripe;  it  is  easily  seen  in  a 
freshly  cut  brain,  appearing  as  a  white  line  in 
the  cortex. 

5.  Nuclei  of  the  Cerebral  Nerves 

1.  The  olfactory  nerve  (Nerve  I)  cf.  page  466. 

2.  The  optic  nerve  (Nerve  II)  cf.  visual 
paths  (Fig.  119). 

3.  Nerves  of  the  eye  muscles:  the  oculo- 
motor nerve  (Nerve  III),  the  trochlear  nerve 
(Nerve  IV),  the  abducens  nerve  (Nerve  VI)  are 
all  purely  motor. 

The  nucleus  of  the  oculomotor  nerve  lies  in 
the  region  of  the  anterior  quadrigeminal  bodies, 
in  the  floor  of  the  aqueduct  of  Sylvius.  It  has 
a  medial  division,  lying  exactly  in  the  middle 
line,  which  sends  fibres  to  both  sides,  and  a 
larger  lateral  one,  on  each  side,  which  also  sends 
off  fibres  to  the  opposite  side.  The  nucleus 
consists  of  aggregations  of  cells,  each  of  which, 
probably,  supplies  a  definite  muscle.  The  roots 
issue  from  the  inner  edge  of  the  cerebral  pe- 
duncle, in  front  of  the  pons,  the  fibres  pierce 
the  dura  mater,  run  into  the  wall  of  the  caver- 
nous sinus,  and,  through  the  superior  orbital 
fissure,  reach  the  eye.  The  nerve  is  divided 
into  a  superior  and  an  inferior  division  (ramus 
superior  and  inferior).  From  this  latter  a  short 
branch,  the  radix  brevis  ganglii  ciliaris,  goes  to 
the  ciliary  ganglion  (Fig.  120). 

4.  The  nucleus  of  the  trochlear  nerve  lies  in  the 
caudal  extension  of  the  oculomotor  nerve  nucleus 
in   the    region   of   the  posterior  quadrigeminal 


Fig.  118. — The  giant  pyramidal  type  in  the  anterior  central 
^convolution.     (After  Brodman.) 


NUCLEI  OF  THE  CEREBRAL  NERVES 


459 


bodies.  The  fibres  pass  from  the  nucleus  dorsally  and  decussate  completely 
in  the  velum  medullare  anticum.  They  appear  back  of  the  quadrigeminal 
bodies,  pass  around  the  cerebral  peduncles  towards  the  front  and  ventrally, 
and  reach,  through  the  wall  of  the  cavernous  sinus  and  the  superior  orbital 
fissure  (Fissura  orbitalis  superior) ,  the  superior  oblique  muscle.  (Musculus 
obliquus  superior.) 

5.  The  Abducens  Nerve. — The  nucleus  lies  in  the  region  of  the  pons,  in 
the  floor  of  the  fourth  ventricle.  The  fibres  pass  ventrally,  emerging  between 
the  pons  and  the  pyramid.  The  nerve  passes  through  the  cavernous  sinus 
and  the  superior  orbital  fissure  to  the  external  rectus  muscle.  (Musculus 
rectus  externus.)     (Fig.  121.) 

Connections  between  the  muscles  of  the  eye,  as  well  as  with  the  Deiter 
nucleus  (of  the  vestibular  nerve)  and  the  cerebellum  are  made  by  the  posterior 
longitudinal  bundle  (Fascicul.  longitud.  post.),  which  can  be  traced  from  the 


Corp.  Quadrigemina 


Pons 


Fig.  119. — Schematic  view  of  the  location  of  the  nuclei  Nr.  III-XII.  Motor  nuclei  dotted, 
sensory  deep  black.  XI X  means  X  and  IX  (common  nucleus  of  Nr.  IX  and  X).  (After 
Villiger.) 


posterior  commissure  to  the  spinal  cord,  and  which  among  other  things 
produces  the  relations  existing  between  ocular  movements  and  changes  in 
equilibrium. 

6.  The  trigeminal  nerve  (Nerve  V) ,  a  mixed  nerve,  has  a  very  large  nucleus. 
The  main  motor  nucleus  lies  in  the  dorsolateral  tegmental  part  of  the  pons. 
In  addition,  there  is  a  smaller  motor  nucleus  in  the  quadrigeminal  region  at 
the  side  of  the  aqueduct.  The  motor  root  passes  directly  to  the  third  branch 
of  the  mandibular  nerve. 

The  thicker  sensory  root  has  its  origin  in  the  Gasserian  ganglion,  enters 
the  pons,  and  runs  near  the  sensory  end  nucleus,  where  the  fibres  divide  into 
ascending  and  descending  branches. 

The  ascending  branch  ends  in  a  collection  of  cells,  situated  next  to  the 
motor  nucleus  in  the  tegmental  part  of  the  pons  (nucleus  sensibilis  N.  V) ; 


460 


DISEASES  OF  THE  BRA IX 


the  descending  branch  ends  in  a  cell  column  which  is  the  caudal  extension  of 
the  before  mentioned  sensory  nucleus.  This  collection  of  descending  fibres 
is  called  the  descending  or  spinal  trigeminal  root;  it  can  be  traced  to  the 
second  cervical  segment.  The  caudal  part  of  this  sensory  nucleus  is  identical 
with  the  gelatinous  substance  of  Rolando  in  the  posterior  horn  of  the  spinal 
cord. 

The  facial  nerve,  a  motor  and  secretory  nerve  (N.  VII) .     The  nucleus  lies 
in  the  tegmental  part  of  the  pons,  ventrolaterally  from  the  abducens  nucleus. 


Fig.  120. — Schematic  cut  behind  the  anterior  quadrigeminal  bodies  through  the  nucleus  of 
the  oculomotor.     Instead  of  nucleus  Nr.  IV  read  Nr.  III.      {After  Edinger.) 


The  fibres  pass  dorsally  around  the  nucleus  of  the  abducens  nerve,  then 
ventrally  and  appear  at  the  posterior  edge  of  the  pons,  at  the  side  of  the  olive. 
The  intermediate  nerve  (X.  intermed.  Wrisbergii),  classified  as  a  minor 
part  of  the  facial  nerve  because  so  closely  associated  with  it,  has  partly  sen- 
sory functions  (fibres  of  taste) ,  partly  secretory  (through  the  chorda  tympani) . 
Its  sensory  fibres  from  the  geniculate  ganglion  end  probably  in  the  sensory 
end-organ  of  the  glosso-pharyngeal  nerve. 


NUCLEI  OF  THE  CEREBRAL  NERVES 


461 
(See 


The  auditory  nerve  (nervus  acusticus,  N.  VIII),  cf.  auditory  paths. 
Fig.  122.) 

The  glosso-pharyngeal  nerve  (N.  IX),  a  mixed  nerve.  The  motor  fibres 
arise  in  two  nuclei: 

1.  The  dorsal  nucleus  in  the  floor  of  the  fourth  ventricle,  and 

2.  The  ventral  nucleus  (or  ambiguus) ,  dorsal  from  the  olive. 

The  sensory  fibres  arise  in  both  ganglia  of  the  nerve  (G.  superius  and 
petrosum)  and  pass  as  sensory  roots  into  the  medulla  oblongata  beneath  the 
facial  and  auditory  nerves  behind  the  olive. 

Here  the  fibres  divide  into  ascending  and  descending  branches.  The 
ascending  end  in  the  nucleus  ala  cinerece,  the  descending  (the  so-called 
tractus  solitarius)  in  the  adjoining  nucleus  tractus  solitarii. 


Fig.  121. — Cross-section  in  the  region  of  the  origin  of  the  abducens.     {After  Edinger.) 

The  pneumo gastric  nerve  (Nervus  vagus,  N.  X),  a  mixed  nerve.  The 
motor  fibres  arise  in  the  same  nuclei  as  those  of  the  glosso-pharyngeal  nerve, 
that  is  in  the  nucleus  dorsalis  and  in  the  nucleus  ambiguus.  The  sensory  fibres 
arise  in  both  ganglia  of  the  vagus  nerve  (G.  jugulare  and  nodosum),  pass 
below  the  ninth  nerve,  back  of  the  olive  into  the  medulla,  and  end  in  the 
same  sensory  end  nuclei  (nucleus  alae  cinerea?  and  nucleus  tractus  solitarii) 
as  those  of  the  glosso-pharyngeal  nerve. 

The  spinal  accessory  nerve  {nervus  accessorius,  N.  XI),  a  pure  motor 
nerve.  It  rises  from  a  collection  of  cells  that  begins  in  the  caudal  extension 
of  the  nucleus  ambiguus  (cerebral  part  of  the  accessory  nucleus)  and  can  be 
traced  to  the  seventh  cervical  segment  (spinal  part  of  the  accessory  nucleus). 


462 


DISEASES  OF  THE  BRAIN 


The  latter  is  situated  at  the  base  of  the  lateral  horns,  and  in  the  dorsolateral 
part  of  the  anterior  horns  of  the  spinal  cord. 

From  this  nucleus  arise  9-13  root-fibres,  which  leave  the  medulla  oblon- 
gata, resp.  the  cervical  white  matter  (Fig.  124). 

The  hypoglossal  nerve  (nervus  hypoglossus,  N.  XII),  a  pure  motor  nerve. 
The  nucleus  lies  in  the  floor  of  the  fourth  ventricle,  next  to  the  raphe.  The 
fibres  pass  ventrally  and  leave  the  medulla  in  10-15  root-fibres,  between  the 
olive  and  pyramid  (Fig.  125). 


S^p-^Ui-jn^f^- — -, 


Fig.  122. — Motor  nucleus  and  root  of  the  trigeminal.     (After  Edingcr.) 


6.  General  Relation  between  Structure  and  Function  of  the  Brain 

(a)  Theory  of  Projection;  Paths  and  Fields  of  Projection 

The  cortex  receives  stimuli  from  the  sensitive  parts  of  the  surface  of  the 
body  (the  entire  skin,  joints,  muscles,  so-called  higher  sensory  surfaces,  as 
retina,  organ  of  Corti,  etc.),  and  sends  out  stimuli  into  parts  that  may  be 
moved  volitionally,  to  the  muscles,  and  to  certain  glands. 

The  paths  which  connect  the  cortex  with  the  periphery  are  called  pro- 
jection paths. 

There  is  no  uninterrupted  connection  between  the  cortex  and  a  sensory  or 
muscular  end-organ.  Between  the  cortex  and  periphery  gray  masses  (e.  g.,  of 
the  spinal  cord)  are  interposed.  In  the  cells  of  these  interposed  gray  masses 
the  sensory  nerves  end  and  the  motor  arise. 

The  cells  of  these  gray  masses  are  connected  with  the  cortex  through  ascend- 


THEORY  OF  PROJECTION:  PATHS  OF  PROJECTION        463 

ing  paths.  There  are  then  in  the  projection  paths,  between  periphery  and  cor- 
tex, one  or  more  gray-matter  way-stations.  So  all  sensory  nerves  of  the  trunk  and 
the  limbs  starting  from  the  periphery  have  their  first  end  in  the  gray  matter 
of  the  spinal  cord  or  of  the  medulla  oblongata  (sensory  end-nuclei).  And 
all  motor  nerves  for  trunk  and  limbs  have  their  origin  in  the  gray  matter  of  the 
spinal  cord  (anterior  horn  cells,  motor  origin  cells). 

But  the  cerebral  nerves  are  wholly  analogous  to  these.  They,  too,  have 
their  sensory  end-nuclei  and  their  motor  origin  nuclei  in  the  gray  matter 
lying  next  to  the  fourth  ventricle  and  the  aqueduct  of  Sylvius.  These  nuclei, 
too,  are  aggregations  of  ganglia  cells,  which — in  centripetal  directions — 
receive  the  impulses  of  the  peripheral  sensoiy  neurons  and  transmit  them  to 
their  axons  leading  to  the  cortex,  or — in  centrifugal  directions — their  axons, 
themselves,  become  motor  nerves,  passing  to  the  periphery. 

Just  because  this  gray  matter  of  the  medulla  and  the  quadrigeminal 
region  receives  sensory  roots  in  the  end-nuclei,  and  sends  out  motor  roots 
from  origin  nuclei,  one  has  named  this  cerebral  region  in  consequence  of  its 
far-reaching  analogy  with  the  function  of  the  spinal  cord,  the  spinal  cerebral 
region.  This  gray  matter  in  the  floor  of  the  fourth  ventricle  and  aqueduct, 
we  can,  when  taken  together  with  the  nuclei  of  the  posterior  column  of  the 
medulla  and  the  gray  matter  of  the  spinal  cord  (anterior  and  posterior  horns), 
look  upon  as  the  first  station  of  combined  sensory  and  motor  processes,  and 
as  primary  centers  of  these  functions.  These  primary  centers  represent 
the  first  projection  of  the  periphery  in  the  central  nervous  system. 

Any  lesion  of  these  primary  centers  interrupts  the  functional  capacity 
of  some  peripheral  part,  whether  it  be  sensory  or  contractile.  About  the 
term  projection,  see  below. 

For  the  further  connection  of  these  primary  centers  with  the  gray  matter 
of  the  cortex,  we  must  consider  separately,  the  motor  and  the  sensory  system. 

For  the  main  paths  of  motility  (direct  motor  paths),  there  is  no  further 
way-station  between  muscles  and  cortex,  besides  the  subcortical  gray  matter 
we  have  just  mentioned,  which  contains  the  origin  nuclei  of  the  motor  nerves 
in  the  midbrain,  medulla  oblongata  and  spinal  cord.  For  more  detailed 
information  about  these  origin  nuclei  cf.  page  458. 

From  the  cortex  to  these  nuclei  there  is  an  uninterrupted  conduction 
path,  the  central  motor  path. 

Its  region  of  origin  in  the  cortex  represents,  for  the  second  time,  all  vol- 
untary motor  processes  (projection  of  the  second  class,  cortical  center),  in  a 
circuit  still  to  be  described.  For  the  voluntary  movements,  we  have,  then, 
1.  a  cortical  projection,  2.  a  subcortical  projection  (the  latter  for  the  cerebral 
nerves  in  the  gray  matter  of  the  quadrigeminal  bodies,  the  pons,  medulla 
oblongata,  and  for  the  nerves  of  the  trunk  and  limbs  in  the  gray  matter  of 
the  anterior  horn  of  the  spinal  cord).  Both  projection  centers  are  con- 
nected by  motor  projection  paths.     The  main  path  for  the  voluntary  move- 


464 


DISEASES  OF  THE  BRAIN 


ments  of  the  limbs  and  the  trunk  is  also  called  the  pyramidal  tract  (cortico- 
spinal path).  It  rises  in  the  upper  two-thirds  of  the  anterior  central  con- 
volution, and  its  immediate  vicinity,  and  in  the  paracentral  lobule  and 
passes,  without  interruption,  through  the  centrum  semiovale,  the  inner 
capsule  (anterior  two-thirds  of  the  posterior  limb),  through  the  foot  of  the 
cerebral  peduncle  and  the  pons  into  the  medulla,  at  the  lower  end  of  which 
its  greater  part  undergoes  decussation.  The  part  that  has  decussated 
passes  into  the  lateral  tracts  of  the  spinal  cord,  and  ends  in  the  anterior 
horn  on  the  same  side,  from  the  cells  of  which  the  anterior  roots,  the  motor 
nerve  rises.     The  non-decussating  fibres  pass  away  in  the  ventral  bundle, 


tfuc/eus  ofUe 
hferitt  column 


Fig.  123. — Exit  of  the  vagus.     {After  Edinger.) 

and  reach  the  anterior  horn  on  the  same  side.  The  pyramidal  tract  is  the 
longest  of  all  conducting  paths. 

The  corresponding  motor  path  for  the  cerebral  nerves,  the  cortico-bulbar 
path,  arises  in  the  lower  third  of  the  anterior  central  convolution,  passes 
through  the  white  matter,  through  the  genu  of  the  inner  capsule,  the  foot  of 
the  peduncles,  and  ends,  with  much  decussation,  in  the  contralateral  nuclei 
of  the  cerebral  nerves. 

Besides  these  important  direct  motor  paths  there  are  indirect  paths, 
interrupted  in  the  red  nucleus,  and  very  important  in  the  higher  animals,  but 
less  so  in  man.  There  is  no  unanimity  of  opinion  as  yet  as  to  whether  the 
central  path,  that  leads  to  the  red  nucleus,  comes  straight  from  the  cortex, 
or  is  interrupted  in  the  thalamus,  or  whether  it  first  makes  a  large  detour 
about  the  cerebellum,  as  do  the  pyramidal  tracts  which  pass  within  the  tract 
of    the  crusta  through  the  internal  capsule  and  the  cerebral  peduncle  to 


THEORY  OF  PROJECTION 


465 


the  pontile  nuclei  (decussated)  and  into  the  cerebellum  and  through  the 
superior  peduncles  of  the  cerebellum  to  the  red  nucleus. 

Things  are  more  complicated  for  the  centripetal  paths,  as  well  for  general, 
as  for  special  sensation.  Between  the  first  endings  in  the  gray  matter 
of  the  end-nuclei,  and  their  final  endings  in  the  cortex,  if  we  ignore 
further  interruptions,  one  way-station,  at  least,  is  always  interposed;  this  is 
formed  by  the  gray  nucleus  of  the  optic  thalamus  with  its  appendages 
(both  geniculate  bodies)  and  the  gray  matter  of  the  quadrigeminal  bodies. 


fibr-< 


Fig.  124. — Nucleus  and  root  of  the  hypoglossal  nerve,  nucleus  ambiguus  and  root  of  the  vagus. 

(After  Edinger.) 


From  the  sensory  end  nuclei  arises  a  second  centripetal  path,  which,  for 
the  most  part,  passes  into  the  medial  and  lateral  fillet  and  ends  in  the  optic 
thalamus  with  its  appendages  and  in  the  quadrigeminal  bodies.  Therefore 
the  thalamus  and  the  quadrigeminal  bodies  represent  a  second  sensory 
projection.  From  the  thalamus  and  the  quadrigeminal  bodies  arises, 
then,  a  third  path  which  ends  in  the  cortex  of  the  cerebrum,  so  that  the 
cortical  sensory  projection  is  at  least  the  third  projection.  (Cf .  also  page 
491.) 

Although,  according  to  its  embryological  development,  the  ganglia  cells 
of  the  retina  do  not  correspond  to  the  spinal  ganglia,  nor  the  optic  nerve,  to 
a  posterior  root,  but  rather  represents  the  connection  of  two  cerebral  parts 
(the  retina  is  a  cerebral  part  that  has  been  pressed  forward) ,  physiologically 
the  same  observation  that  we  have  just  made  with  reference  to  the  other 
3° 


466 


DISEASES  OF  THE  BRAIN 


centripetal  nerves  may  be  applied  to  the  connection  between  retina  and 
cortex.  Even  the  fibres  of  the  optic  nerve,  which  arise  in  the  cells  of  the 
retina,  find  their  first  ending  in  a  subcortical  nucleus,  its  primary  center, 
only  this  lies  in  the  interbrain  or  midbrain  (the  second  station  of  the  rest  of 
the  sensory  paths),  namely,  in  the  external  geniculate  bodies  of  the  optic 
thalamus  and  the  anterior  quadrigeminal  bodies.  Hence  the  central  path 
goes  through  the  optic  radiation  into  the  cortex  of  the  occipital  lobe  (cf. 
later  visual  pathways).  Here  also  there  is  a  primary  subcortical  and  a 
secondary  cortical  projection. 


.tfudeueX 


Ttbrat  propria^ 
nucUiXn 


.Vucleuj  XII 


Fibrae  afTerefitea 
juLclei  XD 


-UusKniieTLtr. 


Karros  XII. 


Fig.  125. — Nucleus  of  the  N.  hypoglossus  (Weigert  stain). 


The  olfactory  path  demands  separate  treatment.  The  axons  of  the 
peripheral  nerves  end  in  the  olfactory  bulb;  from  here  a  second  path  goes, 
especially  to  the  gray  matter  of  the  olfactory  trigone  and  the  anterior  per- 
forated substance;  from  here  a  third  path,  which  ends  in  the  cortex  of  the 
gyrus  hippocampi  and  in  the  hippocampus  major.  Therefore  for  the  optic 
and  olfactory  nerves,  between  which  and  the  other  sensory  nerves,  in  respect 
to  their  embryological  development,  there  is  not  the  slightest  analogy,  the 


MNEMIC  CORTICAL  FIELDS:  ASSOCIATION  FIELDS         467 

same  law  holds  good,  viz. — that  between  the  periphery  and  the  cortical  center 
gray  matter  intermediate  stations  are  interposed. 

Therefore  we  can  say:  All  the  sensitive  surfaces  of  the  body  and  all  Us 
organs  that  produce  motion,  are  projected  upon  the  cortex  by  the  help  of  one  or 
more  intermediate  stations  of  gray  matter. 

The  word  projection  is  not  to  be  understood  literally,  even  in  its  applica- 
tion to  the  first  projection  in  the  spinal  cord,  and  certainly  not  when  applied 
to  cortical  projections — not  in  the  sense,  that  for  every  point  in  the  periphery, 
there  exists  a  co-ordinate  point  in  the  cortex,  and  that  the  arrangement  of  the 
separate  cortical  elements  is  a  replica  of  that  of  the  peripheral  sensory  and 
muscular  elements.  Such  correspondence  between  periphery  and  cortex 
does  not  exist.  For  instance,  not  each  separate  muscle  has  its  own  center  in 
the  cortex,  but  certain  groups  of  muscles  for  definite  related  movements  are 
represented  there.  [In  other  words  there  is  a  center  for  movements  rather 
than  for  muscles.]  But  in  broad  outline,  the  projection  does  exist,  in  so  far  as 
various  sense-organs,  and  various  sensory  and  motor  processes  of  the  limbs 
are  co-ordinated  with  various  regions  of  the  cortex.  And  even  if,  within  such 
a  cortical  region,  corresponding  to  the  motor  processes,  say,  of  an  upper 
extremity,  the  arrangement  does  not  repeat  the  muscular  scheme  in  absolute 
detail,  on  the  other  hand,  the  entire  region  is  not  as  a  mass  co-ordinated  to  the 
entire  limb,  but  parts  of  it  correspond  to  certain  groups  of  muscles.  In  this 
broader  sense,  the  conception  of  a  projection  of  the  body,  on  the  cortex,  is 
justified. 

Those  cortical  regions,  that  receive  projection  paths,  or  from  which  they 
emerge,  are  called  fields  of  projection.  Their  destruction  is  marked,  there- 
fore, by  the  destruction  of  the  motor  processes  or  sensory  processes  of  defi- 
nite perceptive  or  effector  organs;  their  injury  without  destruction  by  convul- 
sions or  other  phenomena  of  motor  irritation,  or  pains  or  other  sensory 
phenomena  of  irritation  (elementary  hallucinations,  for  instance). 

(b)    The  Mnemic  Cortical  Fields  and  the  Theory  of  Association 

Fields 

The  reception  of  centripetal  stimuli  in  the  projection  fields,  when  this 
innervation  has  reached  a  certain  level, 1  is  the  cause  of  perception.  The  excita- 
tion of  motor  fields  of  projection,  at  a  certain  level  leads  to  innervation  and 
subsequent  movement. 

The  cerebrum  has  another  broader  function — that  of  retaining  lasting 
traces  of  sensory  or  motor  innervations  that  once  existed  (residues  or  rem- 
nants, the  possession  of  the  memory) .     These  remnants  not  only  make 

1  Not  every  stimulation  of  the  cerebrum  is  connected  with  consciousness,  though  it  is  to  be 
assumed,  that,  in  man,  no  consciousness  is  possible  without  corresponding  stimulation  of  the 
cerebrum. 


468  DISEASES  OF  THE  BRAIN 

possible  conscious  recollection  of  perceptions  and  movements,  but  also,  even 
without  leading  to  conscious  reproduction,  as  purely  material  possession  make 
it  possible  for  the  nervous  system,  to  perform  repeated  operations  with  greater 
ease,  certainty  and  perfection;  upon  them  therefore  depend  dexterity. 

This  is  the  mnemic  function  of  the  cerebrum.  On  the  other  hand,  it 
produces  associations  among  these  residues;  this  is  the  associative  function 
of  the  cerebrum.  The  smell  of  a  rose,  for  instance,  rouses  in  consequence 
of  such  association,  a  complete  image  of  a  rose. 

It  has  been  long  debated  whether  the  mnemic-associative  functions  are 
bound  to  the  same  cortical  regions  as  perception  and  innervation,  or  if  they 
have  their  own  peculiar  cortical  fields. 

According  to  a  widely  held  opinion  all  parts  of  the  cortex  receive  pro- 
jection paths,  or  send  them  forth,  so  that  the  entire  cortex  is  divided  into 
projection  fields.  The  elementary  mnemic  possession  belonging  to  every 
projection  field,  that  is,  the  remnants  in  any  one  sense  field,  would,  eventu- 
ally be  localized  within  the  same  cortical  region,  but  may  be  in  other  layers 
as  perception  and  innervation,  so  that,  for  instance,  the  association  among 
elements  of  the  same  sense  fields,  say  among  the  optic  elements,  would  take 
place  in  the  same  cortical  regions,  the  association,  on  the  other  hand,  among 
the  remnants  of  various  sense  fields  (optic,  tactile,  etc.),  would  be  brought 
about  by  the  long  associative  fibres.  Then,  the  same  cortical  region  that 
receives  the  optic  radiation  would  possess  optic  memories.  In  this  view, 
it  is  not  necessary  to  assume  that  the  same  nervous  elements  serve  for  percep- 
tion, innervation,  and  memory,  as  this  is,  indeed,  very  improbable;  the  rem- 
nants might  be  registered  in  the  same  large  cortical  region,  and  yet  be  bound 
to  different  cell-fibre  complexes. 

Opposed  to  this  is  Flechsig's  theory,  which  accepts  only  a  small  part  of 
the  cortex  as  projection  fields.  According  to  him,  only  these  are  in  com- 
munication with  the  periphery  through  in-  and  outgoing  paths.  The  rest 
of  the  cortex  has  no  such  connection  with  the  periphery.  It  is  occupied  by  the 
association  fields,  that  have  connections  only  with  the  projection  fields 
(associative  fibres)  and  with  the  opposite  hemisphere  (commissural  fibres) 
and  carry  on  the  associative  and  higher  mnemic  functions.  Furthermore 
Flechsig  divided  the  entire  cortex,  according  to  the  time  when  myelin  develops 
into  36  different  fields,  of  which  those  first  maturing  (primordial  regions), 
12  in  number,  constitute  the  projection  fields,  the  majority  of  those  maturing 
later  (intermediary  regions),  16  in  number,  and  the  7  which  mature  last 
(terminal  regions)  exclusively  constitute  association  fields  (Fig.  126). 

Flechsig's  projection  fields  include  as  most  important,  both  central  con- 
volutions, beside  the  paracentral  lobe,  the  adjacent  part  of  the  first  frontal 
convolution  and  the  gyrus  fornicatus,  the  transverse  convolution  of  the  first 
temporal  convolution,  with  a  small  part  of  the  latter,  the  vicinity  of  the  calca- 
rine,  also  little  fields  of  the  surface  of  the  occipital  lobe,  the  uncus  of  the 


MNEMIC  CORTICAL  FIELDS:  ASSOCIATION  FIELDS 


469 


hippocampal  gyrus,  and  the  inner  surface  of  the  temporal  poles.     All  the 
rest  of  the  surface  of  the  brain,  and  especially,  both  inferior  frontal  convolu- 


Fig.  126. 


Fig.  127. 
Figs.  126  and  127. — Projection  and  association  fields.  According  to  Flechsig  corona  radiata 
fibres  are  certainly  proved  to  exist  only  in  the  dotted  regions,  not  in  the  regions  not  dotted.  The 
remainder  of  the  first  temporal  convolution  and  of  the  Gyr.  hippocampi  are  still  under  considera- 
tion. The  other  undotted  regions  are,  according  to  Flechsig,  association  centers.  {After 
Flechsig.) 

tions,  the  insula,  the  rest  of  the  temporal  and  occipital  lobes  and  the  entire 
parietal  lobe,  are,  according  to  Flechsig,  association  fields  (Fig.  127). 


470  DISEASES  OF  THE  BRAIN 

In  Flechsig's  theory  two  theses  are  included  that  demand  separate  treat- 
ment: first,  the  purely  anatomical,  that  only  part  of  the  cortex  receives  and 
sends  out  corona  radiata  fibres,  that  only  this  part  is  connected  directly 
with  the  periphery  and  that  the  remaining  portions  of  the  cortex,  are  not, 
therefore,  projection  fields. 

The  second  thesis  is  physiological,  relating  to  the  function  of  the  fields  in 
which  corona  radiata  fibres  are  absent.  These  fields  serve  for  the  association 
between  optic,  tactile,  etc.,  processes,  and,  at  the  same  time,  for  the  higher, 
even  the  highest  psychic  processes. 

So  far  as  the  anatomical  question  is  concerned,  it  must  be  granted  Flechsig, 
that  the  theory  of  equal  distribution  of  projection  fibres  on  the  cortex  can  not 
be  maintained.  The  majority  of  the  most  important  motor  and  sensory  tracts 
actually  crowd  together  into  narrow  regions,  lying  in  and  about  Flechsig's 
projection  fields.  Even  if  the  other  regions  are  not  wholly  free  of  projection 
fibres,  and  this  or  that  boundary  of  Flechsig  is,  as  yet,  disputable,  we  must, 
at  any  rate,  recognize  the  important  separation  of  these  regions  which  are 
very  rich  and  those  which  are  very  poor  in  corona  radiata  fibres,  whereby 
the  fundamental  division  of  Flechsig  must  be  conceded,  at  least  a  relative 
justification. 

A  second  question  is,  whether  these  regions  in  which  corona  radiata 
fibres  are  scanty,  are  really  seats  of  the  highest  associations.  Another  pos- 
sibility is,  that  they  have  elementary  mnemic  functions,  and  as  Exner,  Noth- 
nagel  and  Ziehen  thought,  that  they  are  to  be  regarded  as  memory  fields. 

Then,  in  the  neighborhood  of  every  projection  field,  the  corresponding 
remnants  of  any  one  kind  of  sense  impressions  would  have  their  substratum, 
i.  e.,  in  the  neighborhood  of  the  acoustic  projection  field,  the  acoustic  rem- 
nants, in  the  neighborhood  of  the  optic  projection  field  (medial  side  of  the 
occipital  lobe),  the  optic  remnants  (convexity  of  the  occipital  lobe).  The 
regions  poor  in  corona  radiata  fibres,  would  then  be  mostly  memory  fields, 
nevertheless  the  intra-sensory  associations  would  take  place  within  them, 
which,  as  all  authors  agree,  take  place  within  the  cortex.  But  associations 
among  the  various  sensory  and  motor  areas,  and  all  higher  associative  com- 
plexes, would,  as  Wernicke  supposed,  be  carried  on  through  the  large  asso- 
ciation paths  running  in  the  white  matter. 

For  this  latter  supposition  much  experimental  evidence  may  be  adduced. 

In  the  entire  question,  which  can  by  no  means  be,  as  yet,  satisfactorily 
decided,  the  following  point  of  view  seems  to  be  indicated  as  a  compromise : 

Certain  cortical  regions  richly  supplied  with  corona  radiata  fibres  are 
connected  with  receptor  and  effector  organs :  the  projection  fields. 

There  are  further  cortical  areas  poorly  supplied  with  corona  radiata 
fibres,  without  important  connection  with  the  periphery. 

But  the  distribution  of  perception  and  innervation,  of  memory  and 
association  is  not  such,  that  the  fields  of  the  former  serve  only  for  projection, 


ASSOCIA  TION  PA  THS 


471 


and  only  those  of  the  latter  for  memory,  and  the  latter,  moreover,  for  the 
higher  associations  but  the  fields  of  the  former  kind,  serve  for  retaining  the 
remnants  as  well  as  for  projection. 

The  fields  of  the  latter  kind  serve  almost  exclusively  for  remnants,  hence 
deserve  the  name,  mnemic  fields.  The  association  between  the  remnants 
of  the  same  sense  takes  place  all  over  the  cortex. 

The  associations  between  remnants  of  various  senses,  and  all  higher  com- 
plexes are  made  possible  only  by  the  long  association  fibres.  This  higher 
association  is  bound  to  the  systems,  which  by  association  fibres,  represent 
projection  and  memory  fields  combined. 

Hence,  no  part  of  the  cortex  is  wholly  free  from  remnants.  Granted 
only,  that  certain  parts  serve  as  well  for  projection,  while  others  do  not,  we 
rr  ay  distinguish  the  latter  as  -purely  mnemic  fields,  from  the  projection  fields. 
And  the  higher  associative  processes  are  not  carried  on  in  sepaiate  cortical 
fields  but  demand  the  long  association  fibres,  which  unite  various  cortical 
fields  in  a  common  activity. 

(c)  Association  Paths 

The  short  association  fibres  which  run  within  the  cortex  (intracortical) 
or  unite  neighboring  gyri  (arcuate  fibres),  vary  greatly  in  arrangement  and 
direction  in  different  parts  of  the  cortex. 

The  long  association  fibres,  which  unite  distant  parts  of  the  cortex,  run, 
the  longer  they  are,  the  deeper  into  the  white  substance.  The  knowledge 
we  have  of  them  is  still  very  limited. 

Certain  conspicuous  aggregations  of  long  association  fibres  in  compact 
bundles  have  received  individual  names.  But  one  must  not  forget  that  the 
many,  more  scattered  fibres  with  association  functions,  are  not  included 
therein. 

These  bundles  consist  not  only  of  long  fibres,  which  run  from  beginning 
to  end  in  the  bundle,  but,  in  their  course,  many  fibres  from  the  cortex  enter, 
and  many  pass  out  (Fig.  128). 

Only  the  most  important  pathways  of  the  long  association  fibres  will  be 
mentioned  and  named : 

1.  Uncinate  fasciculus.  Connects  the  orbital  surface  of  the  frontal  brain 
with  the  pole  and  anterior  portion  of  the  temporal  lobe. 

2.  Superior  longitudinal  fasciculus  (or  arcuate).  From  the  frontal  brain 
to  the  parietal-occipital  lobes  and  the  posterior  portion  of  the  temporal  lobe. 

3.  Inferior  longitudinal  fasciculus,  ending  in  the  vicinity  of  the  posterior 
horns,  as  the  outermost  of  the  three  sagittal  medullary  layers,  that  sur- 
round the  posterior  horn  (cf.  Figs.  143  and  144).  It  was  considered  for- 
merly a  mere  bundle  of  association  fibres  between  temporal  and  occipital 
lobes,  but  is  a  mixed  pathway.     It  takes  also  many  projection  fibres  to  the 


472 


DISEASES  OF  THE  BRAIN' 


visual  sphere,  contains  optic  radiations  and  even  corpus  callosum  fibres, 
besides  association  fibres. 

4.  Occipitofrontal  fasciculus,  adjacent  to  the  caudate  nucleus,  a  mass 
of  fibres  also  passing  from  front  to  back. 

5.  Cingulum.  Runs  in  the  white  matter  of  the  gyrus  fornicatus,  begin- 
ning in  the  anterior  part  of  the  frontal  brain,  ending  in  the  occipital  lobe, 
consisting  of  sagittal  fibres,  which,  mostly,  connect  adjacent  cortical  areas, 
but  contain  single  fibres  running  through  its  entire  length. 

6.  The  external  and  extreme  capsules  also  contain  sagittal  association 
fibres.     See  still  other  bundles  of  association  fibres  in  Monakow's  scheme. 

(d)  The  Commissures,  especially  the  Corpus  Callosum 

The  commissural  fibres  connect  cortical  parts  of  both  hemispheres. 
1.  The  most  important  commissure  is  the  corpus  callosum.     The  mass 
of  fibres  included  in  the  center  is  known  as  the  body. 


Fasc.  fronto-centralis 


Regia  Ralandica 

Fasc.  centro-parietalis 

Cingulum 


Fasc.  fronto-occipit. 


Occipital 
pole 


Fasc.  rencinat. 


Cingulum 


Fornix 
Fasc.  longitud.  infer. 


Fig.  128. — Scheme  of  the  most  important  association  paths.'    (After  v.  Monakow.) 

The  anterior  curved  portion  is  called  the  genu  (knee) ;  the  posterior  thick- 
ening, the  pad  or  splenium;  the  middle  part  the  body. 

It  connects,  by  means  of  intra-hemispherical  corpus  callosum  fibres, 
symmetrical  and  asymmetrical  cortical  areas.  This  fibrillation  is  mixed 
with  the  projection  and  association  fibres,  and  constitutes  a  great  part  of 
the  white  matter  (Fig.  1 29) . 

The  knee  (genu)  sends  its  fibres  into  the  frontal  brain,  as  the  anterior 


THE  COMMISSURES 


473 


forceps;  the  splenium  into  the  occipital  and  temporal  lobes  as  the  posterior 
forceps,  the  fibres  of  which,  for  the  most  part,  pass  over  into  the  tapetum  of 
the  posterior  and  inferior  horns  of  the  lateral  ventricle.  The  body  of  the 
corpus  callosum  connects  the  central  parts  of  the  hemispheres,  including 
both  central  convolutions. 

If  one  considers,  that  in  the  centrum  semiovale  projection  and  commis- 
sural fibres  run  side  by  side  (see  scheme),  but  separate  at  the  lateral  edge 
of  the  lateral  horn,  that  then  the  projection  fibres  pass  into  the  inner  capsule, 
whereas  the  commissural  fibres,  forming  the  corpus  callosum,  pass  over  the 
ventricle,  to  the  other  side,  it  is  clear,  that  in  focal  injuries  there  is  a  distinc- 
tion, but  lately  discovered:  a  focal  injur)'  (/,  Fig.  129)  in  the  centrum  semiovale 


Fig.  129. — Scheme  of  the  fibres  in  the  corpus  callosum  (red).  In  the  frontal  plane  only 
connections  of  symmetrical  regions  could  be  indicated.  The  projection  fibres  black.  I,  II, 
Ha  and  b,  III,  foci.  7  in  the  white  matter  affects  fibres  in  tha  corpus  callosum  and  projection 
fibres.  II,  Ha  and  lib  only  fibres  in  the  corpus  callosum.  Ill  in  the  inner  capsule  projection 
fibres  only.     Th,  thalamus;  N.I.,  lenticular  nucleus;  anterior  central  convolution. 

strikes  projection  fibres  and  fibres  of  the  corpus  callosum,  a  focal  injury  in 
the  inner  capsule  {III)  only  projection  fibres,  a  focal  injury  in  the  corpus 
callosum  (II,  Ila,  lib),  only  commissural  fibres.  Since  (see  under  apraxia) 
the  left  hemisphere,  through  the  corpus  callosum,  affects  the  innervations 
of  the  right  hemisphere,  a  focal  injury  (I),  will  cause  besides  paralysis  of 
the  right  extremities,  dyspraxia  of  the  left.  A  focal  injury  in  the  inner  cap- 
sule, on  the  other  hand,  produces  only  paralysis  of  the  right,  in  the  corpus 
callosum  only  dyspraxia  of  the  left  extremities.  Extensive  interruption 
of  the  fibres  of  the  corpus  callosum,  probably  especially  of  the  central  third, 
produces  a  local  symptom:  dyspraxia  of  the  left  upper  extremity — it  does 
not  matter  whether  the  interruption  is  in  the  left  hemisphere,  the  corpus 
callosum  itself,  or  in  the  right  hemisphere.  By  the  great  crowding  of  fibres 
in  the  corpus,  a  lesion  therein  will,  of  course,  be  most  fateful  for  praxia. 
Of  great  importance  in  the  production  of  motor  aphasia,  is  the  interrup- 


474  DISEASES  OF  THE  BRAIX 

tion  of  the  anterior  fibres  in  the  corpus,  since  the  frontal  speech  center 
influences  the  right  brain  centers  of  the  7th  and  12th  nerves  through  the 
corpus  fibres. 

So,  too,  interruption  in  the  splenium  fibres  either  in  it  or  within  the 
forceps,  and  the  white  matter  of  the  temporal  and  occipital  lobes  can  help 
to  cause  alexia  and  mind  blindness  (see  sections  dealing  with  these  subjects). 

2.  The  anterior  cerebral  commissure  connects  the  basal  parts  of  the 
temporal  and  frontal  lobes. 

3.  The  lyre  of  fornix  (Lyra  Davidis)  connects  the  hippocampal  horns. 

7.  Clinical  Localization  in  the  Left  Hemisphere 

Fortunately  pathology,  roughly  speaking,  is  not  seriously  affected  by 
differences  in  the  views  discussed  in  pages  467-472. 

For,  even  if  the  answer  is  left  open,  whether  the  association  between 
various  qualities  (optic,  tactile,  acoustic,  etc.),  is  secured  by  means  of  spe- 
cialized cortical  fields,  orby  long  associative  pathways,  on  one  point  the  adher- 
ents of  both  views  agree,  that  lesions  of  certain  areas  in  the  cerebrum,  if 
cortex  and  white  matter  be  considered  together,  produce  no  massive,  striking 
symptoms  of  lost  functions  in  the  sensory  and  motor  realms,  but  cause 
serious  mnemic  disturbances  and  loss  of  associative  processes,  that,  on  the 
other  hand,  lesions  in  other  areas  are  first  chiefly  characterized  by  paralysis 
or  anaesthesia.  Thus,  destruction  of  the  anterior  central  convolution  causes 
hemiplegia,  of  the  calcarine  region,  hemianopia;  on  the  other  hand,  a  lesion 
in  the  third  frontal  convolution,  the  posterior  third  of  the  first  temporal 
convolution,  of  the  parietal  lobe  and  its  transition  into  the  occipital  lobe 
(in  so  far  as  there  is  no  injury  of  the  pathways  leading  to  the  primary  pro- 
jection centers),  causes  no  paralysis  or  anaesthesia,  but  disturbances  in 
speech,  writing,  reading,  agnosia,  apraxia,  respectively  (cf.  page  518,  ff). 
"Whereas  Flechsig  and  his  school  connect  these  mnemic-associative  disturb- 
ances mainly  with  cortical  lesions,  we  trace  them  mainly  to  injury  of  the 
associative  paths  that  pass  below  the  cortex. 

So  we  can  say  with  some  degree  of  certainty  what  clinical  symptoms  of 
lost  function  will  follow  upon  the  destruction  of  entire  parts  of  the  cerebrum, 
including  cortex  and  white  matter,  i.  e.,  of  entire  lobes,  or  parts  of  lobes, 
though  the  question  as  to  how  far  the  cortical  elements  themselves,  or  the 
association  fibres  passing  through  the  white  matter,  are  implicated,  is,  as  yet, 
in  many  respects,  debatable.  If  one  combines  such  a  purely  clinico-patho- 
logical  localization  with  the  known  results  of  electrical  stimuli  used  to  experi- 
ment upon  motor  areas,  the  following  localization  appears.1 

For  the  more  detailed  localization  of  motor  functions,  the  evidence 

1  Only  those  symptoms  have  been  described,  which  may  be  regarded  as  symptoms  resulting 
directly  from  injuries,  not  conditioned  by  the  effects  of  vicinity  or  distance.  The  entire  survey, 
because  of  its  brevity,  can  naturally  give  only  a  summary  orientation. 


LOCALIZA  TION  IN  THE  LEFT  HEMISPHERE 


475 


obtained  by  means  of  electrical  stimulation,  is  of  the  greatest  importance. 
It  has  been  shown  that,  in  man  also,  the  easily  stimulated  points  are  situ- 
ated, in  overwhelming  majority  in  the  anterior  central  convolution  (Ca),  that 
the  lower  third  belongs  to  the  muscles  of  the  head,  the  middle  third  to  those 
of  the  upper  extremity,  the  upper  third,  together  with  the  paracentral  lobe, 
to  those  of  the  lower  extremity. 

The  posterior  two-thirds  of  the  inferior  frontal  convolution  (possibly 

Ccl4 


■R&£ 


"Raf 


Cca. 


Fig.  130. — The  corpus  callosum  and  its  radiations.  By  breaking  off  with  a  forceps,  the; 
radiation  of  the  posterior  end  of  the  corpus  callosum  is  indicated.  Cc/4,  Splenium;  Tho,  thalamus; 
Tap,  tapetum;  C,  ca  corpus  candicans;  B,  place  where  the  cerebral  peduncle  is  cut;  Fcp,  forceps) 
IV,  optic  nerve;  Raf,  fornix;  Rdf,  Vicq  d'Azyr  stripe;  Fli,  fasciculus  longit.  inf.     {After  Edinger. 


also  the  lower  third  of  the  middle  frontal  convolution)  besides  the  adjacent 
part  of  the  lower  fourth  of  the  central  convolution,  together  with  the  anterior 
part  of  the  island  of  Reil  form  the  frontal  speech  region,  the  destruction  of 
which  causes  motor  aphasia. 

Focal  injuries  at  the  base  of  the  frontal  brain  cause,  if  they  destroy  the 
olfactory  bulb  and  tract,  inability  to  smell  on  the  same  side. 

Destruction  of  the  lower  fourth  of  the  anterior  central  convolution,  alone, 
causes  paresis  of  the  contralateral  muscles  of  the  tongue,  palate,  lips,  cheeks 


476 


DISEASES  OF  THE  BRAIN 


and  of  those  used  in  mastication,  which,  however,  as  all  these  muscles  are 
innervated  from  both  hemispheres,  is  not  very  serious,  and  (except  for  the 
paralysis  of  the  contralateral  genioglossal  muscle)  is  not  permanent.  Con- 
sequently, only  a  moderate  degree  of  dysarthria  remains. 

Serious  paralysis  of  the  muscles  mentioned,  on  both  sides,  as  well  as  of 
those  of  the  throat,  hence,  also  anarthria  or  lasting  serious  dysarthria  only 
appear,  when,  at  the  same  time,  the  corresponding  area  of  the  right  hemi- 
sphere is  destroyed  (cortical  pseudo-bulbar  paralysis). 


p  /  OpR 

Lope- 


,  ope  re. 


Gyn  temp,  prof 


Fig.  131. — Sketch  to  explain  the  effect  of  variously  situated  foci  in  the  left  hemisphere.  The 
blue  lines  indicate  that  subcortical  foci  corresponding  to  the  demarcated  region  cause  the  loss  of 
function  concerned.  1,  Motor  aphasia  (because  of  the  illustration  showing  the  third  frontal 
convolution  in  direct  upward  view  and  not  shortened,  the  frontal  speech  region  appears  unpro- 
portionately  large);  2,  paresis  of  the  lingual,  facial,  masseter,  deglutition  and  laryngeal  muscles 
on  the  opposite  side  (transitory  except  for  the  tongue  muscles) ;  3,  paralysis  of  the  arm  and  the 
hand;  4,  paralysis  of  the  leg;  5,  6,  7,  disturbances  of  sensibility  in  the  face,  arm,  leg  (in  6  also 
paralysis  of  touch) ;  8,  if  destroyed  bilaterally,  deafness — if  on  the  left  side,  pure  word  deafness  (?) ; 
9,  sensory  aphasia;  10,  near  to  the  convexity,  alexia  and  agraphia — if  deeper  near  the  medial 
region,  pure  alexia;  n,  near  it,  amnestic  aphasia — deeper,  apraxia;  12,  amnestic  aphasia;  13,  if 
bilateral,  mental  blindness  (visual  agnosia)  which,  however,  may  occur  also  because  of  various 
other  combinations  of  foci,  besides  amnestic  (especially  optic)  aphasia;  14,  deviation  conjuguee; 
15,  insular  aphasia  (blue) .  P.tr.,  pars  triangulars  of  the  third  frontal  convolution;  P.O.,  pars  orbit- 
alis  of  the  third  frontal  convolution;  P.operc,  pars  opercularis  of  the  third  frontal  convolution; 
Op.R.,  operculum  Rolandi;  S.h.,  S.a.,  S.p.,  ramus  horizontalis,  ascendens,  posterior  fossas  sylvii. 


Lesions  in  the  first  and  second  frontal  convolution  may  disturb,  some 
say,  equilibrium  in  walking,  as  do  lesions  in  the  cerebellum  (frontal  brain 
ataxia). 

Regarding  a  center  for  eye  movement,  to  be  found  in  the  frontal  brain, 
see  page  478. 

The  central  two-fourths  of  the  anterior  central  convolution  contain, 
first  of  all,  the  motor  center  for  the  upper  extremity  on  the  other  side.     From 


LOCALIZATION  IN  THE  LEFTJ HEMISPHERE  477 

above  downwards  occur  in  natural  sequence  the  centers  for  shoulder,  arm, 
hand,  finger. 

The  upper  fourth  of  the  anterior  central  convolution,  and  the  anterior 
part  of  the  paracentral  lobule  lying  on  the  median  surface  contain  the  motor 
center  for  the  legs.  The  posterior  central  convolution,  and  a  part  of  the 
adjacent  superior  parietal  lobe,  is  the  seat  of  sensibility  of  the  opposite  half 
of  the  body  (arranged  from  above  down,  like  those  of  motility)  and  the 
posterior  central  convolution  is  preeminently  the  seat  of  perceptions  of 
movement  and  position,  of  the  sense  of  place  and  space  and  the  corresponding 
concepts.  These  perceptions  and  representations  reach  over  also  into  the 
principally  motor  area  of  the  anterior  central  convolution  (Fig.  131). 

In  contradistinction  to  this,  the  area  for  touch  and  pain  sensations 
extends  over  the  posterior  central  convolution,  backwards,  at  least  in  the  front 
parts  of  the  superior  parietal  lobe.  Destruction  of  the  middle  third  of  the 
posterior  central  convolution  and  the  directly  adjacent  part  of  the  parietal 
lobe  produces  paralysis  of  touch. 

The  middle  third  of  the  superior  temporal  convolution,  together  with  the 
transverse  convolution  of  the  temporal  lobe  (gyrus  transversus  or  profundus) 
represent  the  auditory  center,  the  destruction  of  which,  because  of  the  demi- 
decussation  of  the  acoustic  fibres  causes  deafness,  only  when  it  is  bilateral. 

Destruction  of  the  third  of  the  superior  temporal  convolution,  lying 
behind  it,  and  of  the  anterior  adjacent  parts  of  the  supramarginal  convolu- 
tion causes  sensory  aphasia.  Lesions  in  the  angular  gyrus  cause  disturbances 
in  reading  and  writing  with  difficulty  in  finding  words. 

Lesions  in  the  depths  of  the  angular  gyrus  near  the  median  and  basal 
surface  of  the  brain  cause  merely  disturbances  in  reading  and  difficulty  in 
finding  words. 

Large  lesions  in  the  white  matter  of  the  supramarginal  gyrus,  of  the  angu- 
lar gyrus,  and  possibly  also  in  the  white  matter  of  the  upper  parietal  lobe 
cause,  in  addition  to  these,  apraxic  disturbances.     See  page  544. 

Lesions  at  the  base  of  the  posterior  half  of  the  temporal  lobe  and  the 
adjacent  parts  of  the  occipital  lobe  cause  particularly  great  difficulty  in 
finding  words  (amnestic  aphasia)  (Fig.  132). 

Focal  injuries  in  the  cortex  and  white  matter  of  the  convexity  of  the  occip- 
ital lobe  affect  visual  memories,  the  appreciation  of  forms  and  spatial  rela- 
tions and  the  assimilation  of  visual  sensations.  In  order  to  cause  mind 
blindness,  a  left-sided  focal  injury  will  suffice  in  some  people;  in  the  majority, 
however,  a  bilateral  occipital  focal  injury  is  necessary  to  cause  serious  mind 
blindness. 

Focal  injuries  on  the  median  surface  of  the  occipital  lobe,  which  destroy 
the  vicinity  of  the  calcarine  fissure,  cuneus,  lingual  gyrus,  and,  at  the  base 
the  fusiform  gyrus,  cause  half-blindness. 

Focal  injuries,  which  in  the  white  matter  of  the  occipital  lobe  or  even  of 


478 


DISEASES  OF  THE  BRAIN 


the  parietal  lobe,  include  the  optic  radiation,  cause  half-  or  quarter- 
blindness. 

In  the  angular  gyrus  lies  a  projection  center  for  the  movements  of  both 
eyes  in  the  opposite  direction.  Irritation  there  causes  a  deviation  of  the 
eyes  towards  the  other  side  (the  patient  looks  away  from  the  side  with  the 
focal  injury),  and  frequently,  simultaneous  turning  of  the  head. 

Focal  injuries,  however,  in  the  pons,  between  the  oculomotor  and  abdu- 
cens  nuclei,  cause  paralysis  of  the  "  looking  process"  (Blicklaehmung),  not 
to  be  confused  with  deviation;  the  eyes  can  not  be  led  across  the  middle  line, 
nor  towards  the  side  of  the  focal  injury. 


Fig.  132. — Foci  on  the  median  surface  of  the  left  hemisphere.  15  (red),  Paralysis  of  the  leg; 
16/ disturbance  of  sensibility  in  the  leg;  17,  hemianopia  towards  the  right  side;  18  (in  the  white 
matter)  amnestic  aphasia;  19,  is  said  to  produce  disturbances  in  smelling. 

Paralysis  of  the  center  of  eye  movement  in  the  angular  gyrus  leads  pos- 
sibly, since  from  here  simultaneously  the  inhibition  of  the  antagonists  takes 
place,  to  preponderance  of  contralateral  looks  (the  patient  looks  towards 
the  focally  injured  side).  A  second  center  of  the  eye  muscles  in  the  frontal 
brain,  which  has  been  proved  to  exist  in  higher  animals,  is  assumed  by  some 
authors  to  exist  in  man,  also,  at  the  foot  of  the  first  and  second  frontal 
convolution. 

The  hippocampal  gyrus,  uncus  and  hippocampus  major  are  brought 
into  relation  with  the  senses  of  smell  and  taste,  with  the  latter,  especially  the 
back  part  of  the  fornicatus  gyrus. 

Of  the  functions  of  the  lenticular  nucleus  and  caudate  nucleus  nothing 
certain  is  known;  injury  of  the  optic  thalamus  with  its  appendages,  as  inter- 
mediate station  for  all  sensory  stimulations,  even  for  the  higher  perceptions, 


DIFFERENCES  IN  FUXCTIOX  OF  THE  TWO  HEMISPHERES  479 

causes  serious  disturbances  in  sensibility.  Destruction  of  the  outer  genicu- 
late body,  hemianopia.  Destruction  of  one  of  the  inner  geniculate  bodies 
leads  only  to  serious  auditory  disturbances,  when  the  auditory  path  on  the 
opposite  side  is  injured  somewhere  in  its  course. 

8.  Differences  in  Function  of  the  Two  Hemispheres 

While  for  projection  functions — movement,  sensation,  seeing,  hearing, 
tasting,  smelling — the  right  hemisphere  is  naturally  equal  to  the  left  in  all 
men,  it  takes  a  far  smaller  part  in  the  higher  mnemic  and  associative  func- 
tions. This  holds  good,  at  least,  for  right-handed  people,  ca.  90%  of  the 
total.  The  opposite  is  true  of  the  4  to  5%,  of  left-handed  people.  Both 
hemispheres  are  of  equal  importance  in  the  ambidextrous,  5  to  6%. 

The  right  hemisphere  in  the  right-handed  is  so  far  from  indispensable 
for  functions  of  speech,  that  destruction  of  the  right  frontal  brain,  the  right 
temporal  lobe,  the  right  angular  gyrus,  very  seldom  causes  disturbances  in 
speech,  writing  or  reading,  so  that  these  functions  can  be  fully  performed 
without  the  right  hemisphere. 

On  the  other  hand,  intactness  of  the  right  hemisphere  does  not  protect 
persons  who  have  suffered  extensive  lesions  in  the  left  speech  area,  from 
lingering  speech  disturbances,  that  are  often  serious  and  permanent.  In  the 
course  of  time,  however,  the  right  temporal  lobe  may  vicariously  represent 
and  take  the  place  of  the  left,  and,  to  a  certain  extent,  obliterate  the  aphasic 
disturbances. 

Likewise  the  left  hemisphere  is  more  important  for  praxia;  through  lesions 
in  certain  parts  of  the  left  hemisphere,  not  only  the  right,  but  also,  to  a  cer- 
tain extent,  the  left  upper  extremity,  loses  the  power  to  perform  movements 
from  memory,  to  imitate  movements,  etc.  For  the  simpler  manipulation 
of  objects,  the  memory  movements  of  the  right  hemisphere  suffice,  as  a  rule. 
Even  for  memories  of  form  and  color,  precedence  must  be  assigned  to  the 
left  hemisphere,  and  especially  for  higher  association  of  all  simpler  memo- 
ries, and  the  spontaneous  awakening  of  memories. 

"While  lesions  of  the  central  convolutions  and  of  the  auditory,  visual, 
olfactory  and  gustatory  areas  in  the  right  hemisphere  cause  identically  the 
same  clinical  symptoms  of  lost  function  on  the  left  side,  as  those  of  the 
corresponding  parts  of  the  left  hemisphere  cause  on  the  right  side,  the  right 
frontal  lobe  and  large  parts  of  the  right  temporo-parietal  lobe  are  marked 
as  "silent"  parts  of  the  brain  (lesions  of  the  occipital  lobe  are  noticeable 
through  the  interruption  in  the  optic  radiation  usually  resulting),  by  which 
is  meant,  that  mnemic-associative  disturbances  that  are  caused  by  focal 
injuries  in  the  corresponding  areas  on  the  left  side,  are  only  slightly  notice- 
able when  the  lesion  is  on  the  right  side.  Naturally  with  refinement  of 
methods,  even  the  silent  parts  of  the  brain  will  begin  to  speak,  and  it  must 


480     .  DISEASES  OF  THE  BRAIN 

be  assumed,  that  they,  only  in  a  far  less  degree,  have  a  mechanism  similar 
to  the  corresponding  parts  of  the  left  hemisphere.  When  added  to  left- 
sided  lesions,  the  corresponding  right-sided  lesions  are  clearly  brought  into 
evidence  even  to-day. 

9.  Cerebellum 

The  cerebellum  demands  special  attention.     Cf.  Figs.  114  and  115. 

Of  the  morphologically  important  relations  we  emphasize  only  that  the 
cerebellum  is  connected  with  the  rest  of  the  central  nervous  system  through 
three  pairs  of  crural  connections : 

1.  Crura  cerebelli  ad  cerebrum  or  ad  corpora  quadrigemina,  anterior 
crus  cerebelli,  called  also  superior  cerebellar  peduncle  disappears,  under  the 
quadrigeminal  bodies. 

2.  Crura  cerebelli  ad  pontem,  middle  cerebellar  peduncles,  leave  the 
cerebellum  laterally,  passing  into  the  pons. 

3.  Crura  cerebelli  ad  medullam  oblongatam  or  restiform  body. 
Though  the  physiological  relations  are  still  obscure  in  many  particulars, 

the  following  statements  may  be  assumed  as  probably  true. 

I.  The  cerebellum  receives  its  own  centripetal  tracts,  from  the  periphery 
of  the  body,  and  sends  out  centrifugal  stimulations  to  the  spinal  cord. 

As  centripetal  must  be  named  first  of  all : 

1.  The  lateral  cerebellar  tract:  arises  in  Clark's  column  of  the  spinal  cord, 
and  passes  through  the  restiform  body  into  the  cerebellum. 

2.  Gower's  tract:  arises  in  the  lumbar  portion  of  the  spinal  cord  and 
loses  itself  in  the  cerebellum. 

3.  Other  centripetal  stimulations  come  to  the  cerebellum  from  the 
nucleus  of  the  lateral  tract  and  the  crossed  olive. 

4.  Stimulations  from  the  vestibular  nerve  probably  by  the  help  of  Deiter's 
nucleus. 

By  the  help  of  the  centripetal  tracts,  the  cerebellum  receives  messages 
from  the  muscles  of  the  trunk,  neck,  head,  limbs  (unconsciously  persisting 
analog  of  sensations  of  position  and  movement) ,  and  from  the  labyrinth. 

As  centrifugal  tracts  the  following  must  be  first  considered: 

1.  The  cerebellar  cortex  tract  (roof  nucleus?),  Deiter's  nucleus,  the 
uncrossed  anterior  lateral  funicular  path. 

2.  The  cerebellar  cortex-dentate  nucleus  superior  cerebellar  peduncle — 
red  nucleus — Monakow's  bundle. 

3.  Connections  by  means  of  Deiter's  nucleus  and  posterior  longitudinal 
bundle  with  the  nuclei  for  the  eye  muscles. 

Through  these  centrifugal  pathways  muscles  are  influenced  automatic- 
ally, unconsciously.  The  cerebellum  regulates  automatically  the  holding 
of  the  trunk,  head,  and  eyes,  especially  in  standing  upright  and  walking. 
This  regulation  of  the  cerebellum  does  not  suffice,  in  man,  to  produce  erect 


THE  CEREBELLUM  481 

walking;  the  action  of  the  cerebrum  is  also  essential,  but  it  takes  an  impor- 
tant part. 

The  cerebellum  represents^/w^  an  independent  sense-motor  organ,  which 
by  its  own  centripetal  tracts  {different  from  the  sensory  tracts,  that  go  to  the 
cerebrum,  as  the  posterior  columns,  fillet,  etc.),  receives  impressions  from 
the  periphery,  and  by  its  own  centrifugal  tracts,  makes  use  of  these  impres- 
sions, by  means  of  the  muscles,  particularly  for  the  co-operation  of  the  many 
factors  of  equilibrium,  that  are  concerned  in  standing  upright  and  in  walking. 

II.  But  the  cerebellum  has  (indirect)  connections  with  the  cortex  of  the 
cerebrum,  corticopetal  as  well  as  corticofugal.  Through  the  middle  cere- 
bellar peduncle,  the  corticopetal  tracts,  after  various  interruptions,  reach 
the  frontal  cortex,  from  which  the  corticofugal  tracts  through  the  frontal 
pontile  tract  and  the  pontile  cerebellar  tract  reach  the  opposite  hemisphere 
of  the  cerebellum. 

By  means  of  this  cortico-cerebello-cortical  system,  cerebellar  sensations 
come  to  be  acted  on  in  the  cortex  of  the  cerebrum  which  thus  gains  influence 
over  the  cerebellum.  When  a  frontal  lobe  is  destroyed,  the  volume  of  the 
opposite  hemisphere  of  the  cerebellum  diminishes. 

III.  The  cerebellum,  through  its  superior  peduncles  and  the  red  nucleus, 
sends  other  stimuli  to  the  cortex  of  the  cerebrum,  which  this  answers,  in  part 
at  least,  through  motor  processes,  without  the  assistance  of  the  cerebellum,  by 
the  descending  motor  tegmental  tract,  which  arises  from  the  red  nucleus  and 
ends  decussating  in  the  lateral  corona. 

The  main  symptoms  of  disease  of  the  cerebellum  and  its  peduncles,  or 
of  the  tracts  mentioned,  are: 

1.  Cerebellar  ataxia.     Cf.  page  506. 

2.  Subjective:  vertigo. 

3.  Forced  postures  and  forced  movements,  which  are  observed,  either 
as  an  effect  of  irritation,  or  as  a  symptom  of  loss  of  function,  when  the  hemi- 
spheres of  the  cerebellum  and  especially  the  middle  cerebellar  peduncle 
are  affected,  as  well  as  abnormal  inability  to  move  the  eyes  and  nystagmus. 

4.  The  muscles  of  the  body  of  the  same  side  are  usually  hypotonic  (a 
result  of  the  loss  of  centripetal  stimuli) . 

5.  Occasionally,  in  diseases  of  the  cerebellum,  the  ability  to  carry  out 
antagonistic  movements,  in  immediate  succession  (as  pronation  and  supina- 
tion) is  lost  (diadocokinesis). 

6.  In  contrast  to  the  tabetic  the  limbs  of  many  patients  suffering  from 
diseases  of  the  cerebellum,  after  a  little  wavering,  may  persist  for  a  remark- 
ably long  time  in  one  position;  at  times  even  cataleptic  symptoms  appear. 

7.  A  species  of  dysarthria  that  is  occasionally  observed  (slow,  "scan- 
ning" speech),  could,  if  not  caused  by  pressure  on  the  bulbar  centers  of 
speech,  arise  from  the  loss  of  regulating  influences  from  the  cerebellum 
(analogous  to  a  diadocokinesis). 

31 


482  DISEASES  OF  THE  BRAIN 

8.  One-sided  chorea  appears  in  the  case  of  focal  injuries  that  irritate  the 
superior  cerebellar  peduncle  and  its  connections.  A  host  of  other  symp- 
toms, observed  in  diseases  of  the  cerebellum,  especially  diseases  that  com- 
press the  neighboring  organs  (tumors),  arise  from  effects  on  the  adjacent 
pons,  medulla  oblongata,  etc.,  as  hemiplegia,  spastic  phenomena,  vomiting 
and  looking  palsy  (Blicklaehmung). 

Even  the  direct  symptoms  of  cerebellar  diseases  are  not  always  present 
and  are  often  transitory,  because  there  may  be  compensation,  in  the  course 
of  time,  for  the  losses  concerned.  The  cerebrum  seems  to  be  able  to  com- 
pensate, to  a  great  extent,  for  disturbances,  that  have  arisen  in  the  cere- 
bellum. By  far  the  most  important  and  constant  symptom  is  cerebellar 
ataxia. 

It  is  important  to  note  that  focal  injuries  in  the  cerebellum,  as  opposed 
to  those  in  the  cerebrum,  cause  disturbances,  in  the  majority  of  cases,  on 
the  same  side  of  the  body. 

10.  Brain  and  Reflexes 

In  the  first  place,  the  brain  has  an  inhibitory  influence  on  a  series  of 
reflexes  of  the  other  divisions  of  the  central  nervous  system.  This  influ- 
ence is  evinced  normally  in  the  possibility  of  voluntarily  checking  certain 
reflexes  (coughing,  breathing,  closing  the  eyes  at  the  approach  of  the  hand, 
etc.),  furthermore,  in  the  influence  exerted  on  reflexes  by  mental  disturbances 
(breathing  stopped  by  fear).  In  lesions  of  the  brain,  therefore,  by  loss  of 
inhibition,  many  reflexes  are  exaggerated,  such  as  those  of  the  tendons. 

This,  of  course,  is  true  only  so  long  as  a  remnant  of  connection  between 
cerebrum  and  periphery  is  retained.  When  the  connection  is  absolutely 
broken — total  division  of  the  spinal  cord — exactly  those  reflexes  belonging  to 
the  severed  part  of  the  spinal  cord  are  abolished,  but  in  a  manner  difficult, 
as  yet,  to  understand.  A  few  authorities  still  maintain  that  in  cases  of  a 
total  transverse  lesion  of  the  spinal  cord  above  the  lumbar  swelling  the  knee- 
jerks,  which  at  first  are  abolished,  return  and  are  permanently  exaggerated. 

The  brain  not  only  influences  spinal  reflexes,  but  is  itself  the  center  for 
certain  reflexes. 

1.  Pupillary  reflexes. 

i.  Light  Reflex. — When  light  is  thrown  on  the  eye,  the  pupil  of  that  eye 
contracts  (direct  light  reaction) . 

At  the  same  time,  the  pupil  of  the  other  eye  contracts  (consensual  light 
reaction) . 

2.  Convergence  Reaction. — When  looking  at  near  objects  (convergence  and 
accommodation  reactions)  both  pupils  contract,  more  than  from  light.  This 
must  be  conceived  as  a  concomitant  movement.- 

3.  Orbicular  Phenomenon. — The  contracting  of  the  pupils  that  appears 


PUPILLARY  REFLEXES  483 

when  the  eyes  are  shut  tightly,  must  also  be  regarded  as  a  concomitant  move- 
ment. When  this  is  checked — and  a  series  of  concurrent  factors  does 
destroy  the  effect — this  contracting  becomes  evident. 

4.  Pupillary  Cortical  Reflex. — Contraction  of  the  pupil,  from  mere  direc- 
tion of  the  attention  to  a  source  of  light  that  has  not  been  fixed,  appears  only 
in  few  individuals. 

5.  The  asserted  contracting  of  the  pupil  from  a  mere  concept  of  a  bright 
object,  has  been  proved  doubtful  by  further  investigation. 

6.  Psycho-reflex. — On  the  other  hand,  without  doubt,  a  dilatation  of 
the  pupils  occurs,  not  only  with  all  sorts  of  painful  or  vigorous  stimulations 
(especially  of  the  skin,  but  also  from  noises)  but  with  every  psychic  disturb- 
ance (anxiety,  terror)  and  energetic  mental  work  or  strongly  concentrated 
attention. 

During  the  vivid  conception  of  a  dark  object  the  dilatation  of  the  pupil 
appears  as  such  a  psycho-reflex,  which,  however,  is  independent  of  the 
content  of  the  concept  (anything  dark)  and  the  result  only  of  concentrated 
attention. 

In  sleep  maximum  contraction  of  the  pupils  occurs. 

Anatomical 

The  fibres  of  both  optic  nerves  end,  as  we  see,  half  crossed  in  the  lateral 
geniculate  body  and  the  pulvinar.  Besides  this,  fibres  reach  the  anterior 
quadrigeminal  bodies  (pupillary  fibres).  Hence  arises  a  connection  with 
the  deeply  situated  nuclei  of  the  oculomotor  nerves,  whence  a  stimulation 
of  the  pupil  fibres  causes  contraction  of  the  pupil  (Fig.  133). 

The  tracts  of  the  pupillary  light-reflex,  as  yet  not  wholly  ascertained 
anatomically  (contraction  due  to  light)  are  as  follows : 

1.  Centripetal  limb.  Retina,  optic  nerve,  optic  tract,  anterior  quadri- 
geminal bodies. 

2.  Central  transmission.  Anterior  quadrigeminal  bodies,  nucleus  of 
the  oculomotor  nerve  (probably  not  a  direct  connection) . 

3.  Centrifugal  limb.  Nucleus  of  the  oculomotor  nerve  to  the  ciliary 
ganglion  and  hence  with  the  musculus  sphincter  pupillae. 

Since  in  a  strong  light,  both  pupils  contract  (consensual  contraction  of 
the  unexposed  eye),  there  must  be  a  connection  between  each  anterior 
quadrigeminal  body  and  both  oculomotor  nuclei. 

The  dilatation  of  the  pupils  may  result  from  the  innervation  of  the  dila- 
tator pupillae  by  the  sympathetic  nerve. 

The  center  is  Budge's  centrum  ciliospinale  in  the  spinal  cord,  lying  at 
about  the  level  of  the  origin  of  the  first  dorsal  nerves.  It  receives  its  stimula- 
tions from  the  skin  through  the  posterior  roots,  also  from  other  sources  and 
sends  the  innervation  through  rami  communicantes  to  the  superior  cervical 


484  DISEASES  OF  THE  BRAIN 

ganglion  of  the  sympathicus,  hence  to  the  Gasserian  ganglion,  and  through 
the  ramus  ophthalm.  trigem.  to  the  long  ciliary  branches  and  to  the  iris. 

But  dilatation  of  the  pupils  through  the  dilatator  pupillae,  that  is  through 
the  stimulation  of  the  sympathetic  nerve,  plays  a  smaller  part  than  was 
formerly  believed.  The  dilatation  of  the  pupils  in  all  vigorous  sensory 
irritations,  in  every  psychic  exertion  and  excitement,  in  concentration  of 
attention,  energetic  contraction  of  the  muscles,  etc.,  is  caused  especially  by 
inhibition  of  the  sphincter  center,  i.  e.,  by  the  oculomotor  nerve. 

The  influence  of  psychic  processes  on  the  dilatation  of  the  pupil — which 
can  easily  be  proved,  for  instance,  in  the  case  of  simple  intense  recollection 
by  an  apparatus  for  measuring  differences — points  to  connections  between 
the  cortex  and  the  sphincter  center  (Cf),  probably  also  with  the  ciliospinal 
center. 

Pathology  of  the  Pupillary  Reflexes 

When  a  lesion  destroys  any  part  of  the  reflex  arc,  the  pupillary  reflex  is 
destroyed.  The  peculiar  conditions,  under  which  total  loss  of  pupillary 
contraction  and  dilatation,  especially  when  exposed  to  light,  slowness  in 
moving  the  pupils,  hippus,  hemianopic  reaction  of  the  pupils,  myosis,  my- 
driasis, anisocoria  (difference  in  the  diameter)  appear,  are  discussed  in  a 
special  chapter.     (See  also  General  Symptomalogy.) 

Loss  of  the  psychic-reflex  alone,  is  found  frequently  in  certain  mental 
diseases  (juvenile  idiocy).  The  assumption  of  a  so-called  "paradoxical" 
pupillary  reaction  (dilatation  in  light),  is  founded  in  the  majority  of  cases 
upon  a  dilatation  that  quickly  follows  an  unnoticed  contraction. 

II.  Eyelid  reflexes. 

(a)  Touch  reflex:  closure  of  the  eye  when  the  cornea  or  conjunctiva  is 
touched  (trigeminal  nerve,  nucleus  of  the  facial  nerve  and  this  nerve  itself) . 
It  is  absent  in  diseases  of  the  pons,  on  both  sides  also  in  hysteria. 

(b)  Light  reflex:  closure  of  the  lid  on  sudden  exposure  to  strong  light, 
probably  caused  through  the  cortex  (absent  in  cortical  blindness) . 

III.  Palatal  or  choking  reflex,  as  well  as  the  reflex  act  of  swallowing: 
sensory  divisions  in  the  pneumogastric  and  glosso-phary ngeal  nerves .  Center : 
the  nucleus  of  the  motor  trigeminus,  the  pneumogastric,  glosso-pharyngeal 
and  hypoglossal  nerves.     Centrifugal  division:  these  nerves. 

IV.  Vomiting. — Sensory  division:  pneumogastric,  glosso-pharyngeal, 
splanchnic  nerves.  Center  in  the  medulla.  Motor  division:  pneumogas- 
tric, splanchnic  and  phrenic  nerves. 

(a)  Coughing  and  sneezing  made  possible  through  the  fifth,  ninth,  tenth, 
eleventh  and  twelfth  nerves. 

We  may  only  suggest  the  importance  of  the  medulla  for  the  automatic 
regulation  of  the  heart  and  respiratory  innervations,  and  of  a  vaso-con- 
strictor  center  supposed  to  exist  in  the  medulla  oblongata. 


SECRETORY  FUNCTIONS  OF  THE  BRAIN 


485 


11.  Secretory  Functions  of  the  Brain 

1.  For  secreting  the  sweat,  a  center,  including  the  spinal  centers,  is  sup- 
posed to  be  in  the  medulla  oblongata. 

2.  Secretion  of  the  Saliva.— There  is  in  the  medulla  oblongata  a  reflex, 
salivary  center,  which  controls  the  chorda  tympani,  as  well  as  the  sympa- 
thetic secretory  nerves  of  the  salivary  glands  (in  the  passing  of  the  pons 
into  the  medulla,  in  the  tegmentum,  the  so-called  nucleus  salivatorius).  In 
bulbar  paralysis,  excessive  flow  of  saliva  occurs. 


Dilatator     gpAinctet- 


Ggicitia 


DitaTa-tor 


Ggi-  Suprem 
$ymp. 


'lc  radiation 


Fig.  133. — Simplified  scheme  of  pupillary  reflexes.  Cf,  Presumed  centrifugal  path  from  the 
cortex  to  the  center  of  the  sphincter;  Ggl.  gen.  lat.,  ganglion  geniculatum  laterale;  N.N.  III., 
nucleus  N.  oculomot;  N.III.,  nervus  oculomot. 

Even  if  the  salivary  secretion,  caused  by  taste  and  sensory  stimulations 
of  the  oral  cavity  (trigeminous  nerve)  is  a  reflex  of  the  medulla,  the  salivary 
secretion  that  takes  place  at  the  sight,  smell,  and  imagination  of  food  (the 
"mouth  waters")  must  be  called  a  cortical  reflex. 

3.  Secretion  of  Tears.- — The  subcortical  reflex  has  its  center  in  the  medulla 
oblongata.  Centripetal  fibres  run,  especially  in  the  trigeminus,  and  other 
sensory  nerves,  centrifugal  in  the  facial  nerve.  The  secretion  of  tears  is 
under  cortical  influence,  as  is  proved  by  its  dependence  on  psychic  processes. 

4.  The  medulla,  through  the  splanchnic  nerves,  exerts  also  an  influence 
on  the  secretion  of  the  liver  (glycosuria  in  diabetic  puncture). 


486  DISEASES  OF  THE  BRAIN 

II.  LOCAL  SYMPTOMATOLOGY 
i.  General  Symptoms 

i.  Disturbances  of  consciousness. — In  the  first  place  there  are  various 
degrees  of  a  general  diminution  of  consciousness.  In  coma—ior  instance, 
in  an  epileptic  fit,  or  often,  after  an  apoplectic  attack — there  is  total  loss  of 
consciousness. 

The  reflexes  are  altogether,  or,  for  the  most  part,  abolished.  The  limbs, 
when  held  up,  fall  limply  down,  if  left  to  themselves.  The  comatose  patient 
.can  not  be  roused  however  strong  the  stimulus  may  be. 

Slighter  degrees  of  lessening  of  consciousness  are  called  sopor  and  somno- 
lence. The  soporose  patient  is  roused  by  strong  stimuli  but  only  for  the 
time  being;  the  somnolent  person,  is  rather  easily  aroused  and  capable  of 
reactions,  but  when  left  to  himself,  easily  falls  back  into  a  drowsy  condition, 
in  which  most  external  occurrences  have  no  existence  for  him. 

Associated  with  these  diminutions  of  consciousness,  appear  irritations 
of  and  changes  in  the  conscious  processes. 

These  dimmings  of  consciousness,  with  their  countless  sense  delusions, 
and  the  ensuing  motor  restlessness,  are  designated  as  delirium.  Disturb- 
ance of  consciousness  together  with  sense  delusions  causes  disorientation. 
Delirium  appears  in  meningitis,  infectious  diseases,  in  the  course  of  alcohol- 
ism, morphinism,  and  also  after  occlusion  and  other  lesions  of  vessels. 

Anxiousness,  and  torpidity  (stupor),  even  with  catatonic  phenomena, 
are  observed  in  organic  diseases  of  the  brain.  Other  mental  disturbances, 
especially  general  weakness  of  memory  (loss  of  or  difficulty  in  rousing  the 
formerly  associated  mnemic  possessions) ,  disturbance  in  the  ability  to  notice 
(inability  to  assimilate  anything  new  into  the  memory),  disturbances  in 
attention,  judgment,  feeling,  volition,  supersensitiveness,  unnatural  suspi- 
cion, increased  to  prejudicial  ideas,  diminution  of  ethical  motives,  narrow- 
ing of  the  circle  of  interest  to  the  Ego,  etc.,  general  symptoms,  which  appear 
frequently,  as  a  result  of  chronic  and  diffuse  disorders  of  the  brain  (arterio- 
sclerosis, progressive  paralysis,  senile  atrophy,  also  multiple  sclerosis,  tumors, 
etc.),  are  described,  in  more  detail,  in  the  text-books  of  psychiatry. 

2.  Headache  is  a  very  ambiguous  symptom,  which,  even  when  the  pain 
is  clearly  localized  by  the  patient,  does  not  at  all  warrant  the  conclusion 
that  the  focal  injury  corresponds  to  the  seat  of  pain. 

3.  Vertigo  a  word  used  by  laymen  in  a  very  different  sense — i.  e.,  for  a 
mere  fainting  spell  or  a  darkening  of  the  field  of  vision — means:  (1)  That . 
the  patient's  own  body  or  even  external  objects  seem  to  him  to  move,  at 
least  there  is  disorientation  regarding  his  posture,  or  the  position  of  the 
body  itself,  that  is,  subjective  vertigo  or  (2),  loss  of  equilibrium,  that  is, 
objective  vertigo.     When  severe  the  patient  may  fall.     Vertigo,  especially 


SYMPTOMATOLOGY  OF  CEREBRAL  DISEASE  487 

if  slight  or  if  subjective,  is  seen  in  several  different  conditions,  i.  e.,  anaemia 
of  the  brain,  general  vascular  changes,  cerebral  hemorrhage,  etc.  But  the 
higher  degrees  stand  in  particular  relation  to  cerebellar  and  labyrinthine 
diseases.     (Cf.  pages  481  and  15.) 

4.  Vomiting  is*  a  symptom  of  local  irritation  in  the  medulla  oblongata, 
and  is  classed  among  the  general  symptoms  only  because  this  center  is 
easily  stimulated  to  reaction  by  pressure  and  irritation  in  far  distant  parts  of 
the  brain,  or  perhaps  renexly,  by  disturbances  of  the  sensory  branches  of 
the  dura. 

Cerebral  vomiting  is  distinctly  characterized  by  its  occurring  without 
nausea,  the  ease  with  which  it  occurs,  and  its  occurrence  when  no  nourish- 
ment had  been  previously  taken.     It  is  often  projectile. 

5.  Changes  in  the  pulse,  respiration,  temperature. 

Cerebral  diseases  can  influence,  in  many  ways,  the  action  of  the  heart. 

Slowing  of  the  pulse,  which  frequently  appears  when  there  is  a  rapid  in- 
crease of  pressure  within  the  cavity  of  the  cranium,  by  irritation  of  the 
pneumogastric  center  in  the  medulla  oblongata  is  of  special  importance. 

Even  in  the  absence  of  an  infectious  disease,  a  transient  rise  in  tempera- 
ture occurs  after  arterial  occlusion,  or  cerebral  hemorrhage  or  a  paralytic 
seizure,  without  any  certainty  whatever,  as  to  what  definite  parts  of  the  brain 
are  directly  concerned.  On  the  other  hand,  the  temperature  occasionally 
falls  to  96  degrees. 

Of  the  many  changes  from  normal  respiration,  which  appear  especially 
in  coma,  the  most  important  is  the  Cheyne-Stokes'  respiration,  which  occurs 
also  in  the  final  stages  of  heart  and  lung  diseases  and  uraemia,  and  is  a 
"signum  pessimum."  It  is  marked  by  the  following  series  of  irregularly 
interchanging  phases:  1.  A  number  (20-30)  of  increasingly  superficial 
breaths,  following  one  another  in  quick  succession.  2.  Pause  inbreathing. 
3.  Little  by  little  breathing  beginning  again,  at  first  slow  and  deep,  but 
gradually  increasing  in  rapidity  till  phase  1  is  reached.  This  sequence 
recurs  again  and  again. 

6.  Choked  disc  (merely  a  severe  optic  neuritis)  presents  ill-defined 
boundaries  of  the  turgid,  reddish,  and  swollen  papilla,  with  total  dis- 
appearance of  its  boundary,  often  with  hemorrhage  and  white  spots, 
enlarged  and  tortuous  veins,  and  constricted  arteries;  the  vessels  seem  to 
be  broken  off  at  the  edge  of  the  disc.  Choked  disc  is  a  general  symptom, 
because  it  occurs  in  consequence  of  any  space-restricting  lesion  anywhere 
within  the  cranium  (especially  in  tumors,  internal  hydrocephalus,  abscesses) , 
also  in  meningitis,  less  often  in  cerebral  syphilis  and  lead  poisoning  and  very 
seldom  in  multiple  sclerosis.  It  occurs  most  frequently  with  tumors  of  the 
posterior  cranial  fossa.  It  is  most  frequently  bilateral,  even  when  appearing, 
as  it  often  does,  on  one  side  first. 


488  DISEASES  OF  THE  BRAIN 

2.  Projection  Disorders 

Disorders  in  Motion  and  Sensation 

We  differentiate: 

i .  Projection  Disorders. — They  affect  movement,  sensation,  and  secretion. 
Phenomena  of  irritation  or  loss  of  functions  of  receptor  or  effector  organs. 

2.  Mnemic-associative  Disturbances. — They  affect  those  functions  of  the 
brain  which  consist  in  storing  up  past  stimulations,  and  in  fixing  their 
connection.  The  associative  work  of  the  brain  does  not  correspond  to  any 
arrangements  of  the  sense  and  muscle  apparatus  of  the  body,  but  to  the 
connections  established  by  experience;  so,  for  instance,  the  association  between 
the  scent  and  the  appearance  of  the  rose,  is  not  at  all  brought  about  by  the 
connection  between  the  mucous  membrane  of  the  nose  and  the  retina. 

The  projection  disturbances  are  divided  into  motor,  sensory  and  secre- 
tory, and  each  of  these  groups,  into  symptoms  of  irritation  and  of  loss. 

(a)  Motor  Symptoms  of  Irritation 

Cortical  spasms.  As  the  electric  current,  and  all  other  irritants  that 
influence  the  motor  cortical  centers,  cause  contractions  in  the  muscles  of 
the  limbs  on  the  other  side  of  the  body,  so  with  pathological  irritants  (tumors, 
hemorrhage,  scars,  etc.),  spasms  occur  in  the  limbs.  Usually  the  spasm 
is  first  tonic,  later  clonic. 

According  to  the  place  in  the  cortex  that  is  affected  by  the  irritation, 
the  muscles  of  the  face,  arm,  or  leg  are  the  seat  of  the  spasm.  When  the 
irritation  is  lasting  and  extended,  the  spasm  spreads  from  the  region  first 
attacked  to  the  other  limb  on  the  opposite  side  and  it  proceeds  in  such  a 
way,  that  the  muscles  are  affected,  in  the  order  in  which  their  cortical  centers  are 
arranged,  cf.  page  475,  so  that  the  spasm  that  has  started  in  the  face,  continues 
first  in  the  upper,  then  in  the  lower  extremities.  Finally,  the  spasm  can  pass 
over  to  the  same  side  of  the  body,  in  which  the  focal  injury  lies,  whereby  the 
clinical  picture  of  a  general  epileptic  fit  is  produced.  Even  when  the  spasm 
does  not,  universally,  attack  the  other  side,  the  usual  bilateral  muscle  groups 
(of  the  jaw,  for  breathing,  etc. — viz.,  in  what  is  otherwise  one-sided  spasm) 
are  also  affected  by  twitching,  even  though  they  lie  on  the  other  side. 

These  cortical  spasms,  confined  to  one  group  of  muscles,  or  to  one  side 
of  the  body,  or  at  least,  beginning  locally,  are  known  as  Jacksonian  or  cor- 
tical epilepsy.  They  are  contrasted  with  the  convulsions  of  ordinary  epilepsy, 
which  affect  the  whole  body  from  the  start. 

After  severe  Jacksonian  convulsions,  paresis  appears  in  the  muscles 
attacked,  as  a  consequence  of  exhaustion,  but  retrogresses  after  a  few  hours, 
at  most.  But  if  in  the  progress  of  the  disease,  as  often  happens,  though  the 
effect  of  irritation  is  only  temporary,  the  lesion  causing  the  spasms,  e.  g.,  a 


MOTOR  SYMPTOMS  OF  IRRITATION  489 

neoplasm  leads  to  destruction  of  the  motor  cortical  center,  permanent  pa- 
ralysis of  those  groups  of  muscles,  which  were  formerly  affected  by  the  spasm, 
appears.  Together  with  the  extremities,  on  the  opposite  sides,  head  and 
eyes  fall  into  a  mostly  tonic  spasm.  If  the  irritating  focal  injury  lies  towards 
the  left,  both  eyes  and  the  head  turn  towards  the  right:  conjugate  deviation 
(deviation  conjugue).  In  this  deviation,  produced  by  an  irritative  cause, 
the  patient  looks  towards  the  side  on  which  the  limbs  are  spastic,  contrary 
to  the  deviation  caused  by  paralysis.  It  is  worthy  of  notice,  that  not  only 
the  contralateral  eye,  but  both  eyes  are  affected  by  the  effect  of  this  irritation 
which  exists  in  one  hemisphere  alone. 

This  combination  of  tonic-clonic  spasms  in  isolated  groups  of  muscles 
on  one  side,  can  occur,  pathologically,  only  with  cortical  irritation.  It 
must  be  remembered,  however,  that  focal  injury  not  immediately  in  the  center 
concerned,  hit  adjacent  to  it,  can  cause  the  irritation. 

Therefore  spasm  is  of  much  less  importance  in  the  diagnosis  of  the 
localization  of  disease  than  is  palsy.  Symptoms  of  lost  function  are  of  much 
greater  surety,  for  local  diagnosis,  than  those  of  irritation. 

General  spasms,  and  especially,  tonic  spasms  can  also  be  discharged 
from  deeper  lying  subcortical  centers.  In  irritation  of  cortical  motor  centers 
(Jacksonian  epilepsy),  the  characteristics  are:  spasmodic  contractions,  con- 
fined to  a  group  of  muscles,  or  at  most,  to  one  side,  the  change  from  tonic  to 
clonic,  and  its  spreading  according  to  the  location  of  the  centers  for  indi- 
vidual limbs,  in  the  anterior  central  convolution. 

Choreic  Twitchings .     Hemichorea 

Movements  of  the  limbs  much  resembling  those  of  Saint  Vitus  dance  in 
children  and  parturient  women,  appear  in  focal  injuries,  on  one  side,  in  par- 
etic limbs  (often  long  after  the  paralysis),  or  sometimes  precede,  by  a  con- 
siderable time  a  paresis  or  paralysis.  {Hemichorea.)  If,  as  is  usual,  they 
appear  in  hemiplegic  limbs,  we  have  posthemiplegic  chorea.  The  affected 
limbs  are  often  hypotonic.  The  hemichorea  is  sometimes  accompanied  by 
pain.  The  kind  of  disturbance  in  movement  varies  in  different  cases, 
sometimes  resembling  mere  tremor,  at  others  athetosis. 

As  to  the  anatomical  basis  of  hemichorea,  as  a  rule  focal  injuries  in  the 
subthalamic  region  (red  nucleus  and  its  connections  with  the  cerebellum 
(superior  cerebellar  peduncle)  and  the  optic  thalamus)  seem  to  be  the  cause. 

Athetosis,  a  mostly  unilateral,  unceasing,  involuntary  movement, 
appearing  in  paralyzed  limbs,  abating  only  in  sleep.  It  consists  in  slow 
spreading,  adducting,  flexing,  and  extending  movements,  especially  of  the 
fingers  and  toes.  (Athetosis  of  the  facial  muscles  is  of  rare  occurrence.) 
Over-extension  of  the  fingers  is  conspicuous.  Athetosis  appears  most 
frequently  in  cerebral  infantile  paralysis,  but  not  until  long  after  the  onset 


49o  DISEASES  OF  THE  BRAIN 

of  the  paralysis,  and  when  it  has,  to  a  certain  extent,  retrogressed.  Athetosis, 
too,  seems  to  be  caused  by  focal  injuries  in  the  sensory  tracts  leading  to  the 
thalamus.  It  may  appear,  exceptionally,  with  diplegic  paralysis,  in  both 
halves  of  the  body. 

The  so-called  double  athetosis  (Athetose  double)  is  a  disease  sui  generis, 
which  appears  symmetrically  on  both  sides  without  preceding  paralysis 
from  an  unknown  cause  and  without  anatomic  concomitants. 

Unilateral  tremor  is  found  occasionally  in  hemiparetic  limbs.  In 
many  cases  it  resembles  perfectly  the  marked  tremor  of  paralysis  agitans. 

Clonic-tonic  twitchings  of  the  palatal  muscles,  150  to  200  in  the  minute, 
have  been  observed,  especially  with  pseudo-bulbar  symptoms,  in  hemiplegic 
patients,  and  seem  to  have  been  traced  back  to  injuries  in  the  vicinity  of  the 
red  nucleus. 

Associated  movements  are  involuntary  movements  appearing  in  other 
limbs  or  in  other  muscles  of  the  same  limb  while  this  limb  is  being  moved 
purposely  or  even  moving  reflexly. 

They  appear  especially  in  the  paralyzed  limbs,  when  strong  movements 
occur  in  the  sound  limbs.  But  also  in  the  sound  limbs,  when  an  attempt 
is  made  to  innervate  the  paretic  ones.  Thus,  when  the  sound  hand  is 
energetically  closed,  an  impulse  discharges  itself  into  the  paralyzed  hand, 
which  can  not  voluntarily  be  closed,  and  it  closes  or  an  unsuccessful  attempt 
to  close  the  paralyzed  hand  has  the  effect  of  making  the  sound  hand  into  a 
fist.  With  sneezing  and  yawning  a  paralyzed  limb  sometimes  extends  or 
flexes. 

StrumpelVs  tibialis  phenomenon;  strong  dorsal  flexion  of  the  foot  while 
the  hemiplegic  leg  is  being  drawn  toward  the  body,  is  an  associated  move- 
ment of  another  group  of  muscles  of  the  same  limb.  Even  in  passive  move- 
ments of  the  sound  limb,  the  paretic  limb  may  sometimes  carry  on  similar 
movements. 

In  paralytics,  chronic  alcoholics,  idiots,  and  aphasics,  we  often  find, 
when  they  are  speaking  associated  movements  of  the  facial  muscles,  especially 
of  the  forehead. 

Contractures  will  be  discussed  later  on  with  the  paralyses.  See  page 
493- 

(b)  Sensory  Irritation  Symptoms 

In  focal  injuries,  that  irritate  the  sensory  tracts  (see  below),  constant, 
or  intermittent  violent  pains  may  appear,  which,  to  distinguish  them  from 
the  usual  pains  produced  by  peripheral  irritation,  are  called  "central  pains." 
These  pains,  sometimes  combined  with  a  feeling  of  heat,  are  felt  in  the 
opposite  side  of  the  body,  or  it  may  be,  too,  in  a  single  limb,  arm,  or  in  the 
face,  tongue,  etc.     Besides  pains,  parassthesias  of  various  kinds,  formication, 


SENSORY  IRRITATION,  PARALYSIS  491 

thermic  sensations,  etc.,  may  appear.  Unilateral  sensations  of  cold  of 
sudden  onset  have  been  noticed,  and  sensations  of  movement,  without 
objective  movement. 

Sensibility  may  remain  undisturbed  in  the  presence  of  these  central  pains; 
but  pains  may  appear  in  regions  insensitive  to  external  irritation — anaesthesia 
dolorosa. 

Sometimes,  hemichoreic  twitchings,  produced  by  focal  injuries,  are 
combined  with  central  pains. 

As  irritation  phenomena,  we  may  regard  the  hyperesthesias  also:  even  a 
slight  touch,  or  a  slight  thermic  stimulus  is  painful,  as  well  as  the  paresthesias 
due  to  touch  alone:  strange,  "funny"  sensations. 

Central  pains  and  parsesthesias  may  be  caused  by  focal  injuries  in  every 
part  of  the  sensory  system,  even  in  the  cortex  and  corona  radiata,  more 
frequently  by  those  in  the  thalamus  and  subthalamic  region.  Therefore 
they  have  no  localizing  value. 

In  the  territory  of  the  special  senses  (sight,  hearing,  etc.),  stimulation 
phenomena  appear  in  the  form  of  elementary  sensations,  the  seeing  of  light, 
flames,  colors,  the  hearing  of  humming,  ringing  and  rustling,  and  of  complex 
hallucinations.  Thus  disease  in  the  occipital  lobe,  may  cause  visual  hallu- 
cinations in  the  hemianopic  half  of  the  field  of  vision. 

(c)  Motor  Symptoms  of  Loss  of  Function 
1.  Paralyses 

Soon  after  a  grave  apoplectic  seizure,  the  so-called  initial  flaccid  paralysis 
appears;  the  passively  raised  limbs  fall  as  if  lifeless. 

The  reflexes  are  lost  at  first,  but  soon  a  certain  tonus  appears  in  the 
muscles,  the  tendon  reflexes  return  and  are  soon  exaggerated,  the  Babinski 
plantar  reflex  appears.  The  stage  of  flaccid  paralysis  usually  lasts  only  a 
few  days,  rarely,  a  few  weeks.  In  some  muscles  a  certain  degree  of  motility 
returns,  e.  g.,  in  those  which  flex  the  arm  and  fingers,  and  now  gradually  the 
flaccid  paralysis  passes  over  into  the  permanent  condition  of  residuary 
hemiplegia,  to  be  described  immediately. 

In  residuary  hemiplegia,  it  is  not,  as  after  complete  division  of  nerves,  a 
question  of  paralysis  in  the  strictest  sense.  The  muscles  are  not  as  in  the 
case  of  nerve  division  robbed  of  all  tonicity,  nor  are  they  incapable  of  reflex 
movements.  On  the  contrary,  in  part  of  the  muscles  hypertonia  appears 
and  the  tendon  reflexes  are  'exaggerated. 

The  behavior  of  the  reflexes,  discussed  on  page  64,  will  be  only  touched 
upon  here. 

In  cerebral  paralyses  a  few  days  after  the  attack  we  find  exaggeration  of 
the  tendon  reflexes  and  mechanical  muscular  irritability.  Often  patellar 
and  ankle  clonus,  less  frequently  hand  and  masseter  clonus  are  found.  Tap- 
ping on  many  other  tendons  (periosteal  spots)  and  muscles,  results  in  in- 


492  DISEASES  OF  THE  BRAIN 

creased  contractions  (supinator,  triceps,  radius-periosteal  reflexes,  etc.). 
In  a  certain  but  not  in  absolute  contrast  to  the  tendon  reflexes  stand  certain 
skin  reflexes.  The  abdominal  and  cremasteric  reflexes  are  generally 
abolished  on  the  paralyzed  side.  But  one  must  consider,  that,  as  a  matter  of 
fact,  even  the  former  can  not  always  be  obtained  in  healthy  people,  and  that 
therefore,  only  a  one-sided  loss  is  significant.  (In  long-standing  hemiplegia 
the  knee-jerk  may  be  greatly  increased  on  the  unparalyzed  side.) 

If  exaggeration  of  the  reflexes  should,  exceptionally,  fail  to  appear,  either 
a  concomitant  disease  of  the  posterior  column  (tabes)  or  neuritis  is  the  cause. 

A  frequent  symptom — in  70-80%  of  cerebral  paralyses — is  the  Babinski 
plantar  phenomenon  (see  page  69).  Oppenheim's  dorsal  leg  phenomenon 
(see  page  69)  and  Kurt  MendeVs  reflex  (plantar  flexion  of  the  toes  instead  of 
the  normal  dorsal  flexion,  when  the  dorsum  of  the  foot  is  tapped,  near  the 
third  and  fourth  metatarsal  bones)  are  frequent  signs  of  paralysis  caused  by 
lesion  in  the  cortico-spinal  path. 

In  the  hemiplegic  limbs,  all  muscles,  to  a  certain  degree,  are  weakened, 
but  some  groups  of  muscles,  the  so-called  preferred  muscles  of  hemiplegia 
remain  strongly  affected  while  the  others  recover  somewhat.  These  pre- 
ferred muscles  belong  to  definite  mechanisms.  The  peculiarity  of  all  cerebral 
paralyses,  is,  that  not  single  muscles,  but  entire  muscular  mechanisms,  i.  e., 
many  muscles  working  together,  called  synergies,  are  paralyzed.  One  must 
consider,  that  a  simple  movement  (such  as  extending  the  index  finger)  is  not 
the  work  of  a  single  muscle.  Antagonists  are  involved  as  well  as  collateral 
and  rotatory  synergists. 

The  mechanisms  particularly  injured  in  cerebral  paralysis  are,  in  regard 
to  the  leg,  all  the  mechanisms  which  help  to  retract  the  leg,  especially  those 
which  bend  the  knee  and  the  dorsal  flexors  of  the  foot.  Because  of  the  weak- 
ness of  the  dorsal  flexion,  the  tip  of  the  foot  often  drags  on  the  ground  and 
the  leg  is  lengthened.  The  extensors  of  the  leg  are  retained,  especially  those 
of  the  leg  below  the  knee  and  the  plantar  flexors  of  the  foot.  Therefore,  the 
leg  may  be  used  stilt-like  in  walking.  In  any  case,  the  leg  is  less  seriously 
affected  than  the  arm.  The  glutasus  medius  is  often  paralyzed,  which  fixes 
the  pelvis  against  the  thigh.  Consequently  in  walking,  the  pelvis  falls  towards 
the  unparalyzed  side.  In  order  to  compensate  for  this,  the  hemiplegic 
patient,  every  time  he  steps  on  the  paralyzed  side,  throws  his  trunk  towards 
that  side.  Hence  occurs  the  peculiar  sideward  movement  of  the  trunk  in 
walking,  found  in  many  hemiplegics.  In  double  paralysis  of  the  gluteus 
medius — a  rare  phenomenon  of  cerebral  infantile  paralysis — a  waddling 
walk  results.     The  body,  at  every  step,  sinks  to  the  side  of  the  standing  leg. 

In  the  upper  extremities,  the  muscles  that  open  the  hand,  and  rotate  it 
and  the  arm  outward,  are  more  seriously  paralyzed,  while  those  of  opposite 
action,  closing  the  hand,  and  inward  rotation  of  the  arm,  are  less  seriously 
affected. 


PARALYSIS;  CONTRACTURES;  HYPERTONIA  493 

The  raising  of  the  shoulder  (M.  cucullaris  and  levator  scapulae)  is  seri- 
ously affected.  The  sternocleido-mastoid  muscle,  on  the  contrary,  is  always 
unaffected.  For  both  upper  and  lower  extremities,  this  rule  holds  good,  that 
the  distal  parts  are  always  more  seriously  affected,  i.  e.,  hand  and  foot,  and 
especially  the  differentiated  movements  of  fingers  and  toes,  the  isolated  move- 
ments in  the  small  joints.  In  contrast  to  these,  there  is  retained  ability  to 
make  a  few  gross  changes  in  the  position  of  the  entire  limb. 

The  frontal  ocular  branch  of  the  facial  nerve  is  spared  (usually,  however, 
the  eye  on  the  paralyzed  side  can  no  longer  be  closed  alone),  whereas  the 
branch  running  to  the  mouth  and  cheek  is  clearly  paretic.  (An  important  dif- 
ference from  the  peripheral  paralysis  of  the  seventh  nerve.)  The  mouth  is 
drawn  to  the  unaffected  side. 

The  tongue,  when  stuck  out,  turns  to  the  paralyzed  side,  because  the 
sound  geniohyoglossus  presses  to  the  other  side,  but  for  the  rest,  is  sufficiently 
mobile. 

Symmetrically  working  muscles,  on  the  two  sides  of  the  body,  which,  as  is 
well  known,  may  be  innervated  by  each  hemisphere  individually  (muscles  of 
the  eye,  back,  larynx,  of  chewing  and  swallowing)  are  unaffected  or  almost 
unaffected  in  hemiplegia. 

The  bilaterally  working  speech  muscles,  too  (of  the  tongue,  palate,  face), 
are  not  so  deprived  of  function  by  one-sided  focal  injury,  that  speech  is  per- 
manently lost.  Serious  dysarthria,  for  the  most  part,  appears  only  as  a  transi- 
tory phenomenon,  after  one-sided  injuries  and  only  when  they  are  left-sided. 
Lasting  anarthria  or  serious  lasting  dysarthria,  appear  almost  only  in 
bilateral  focal  injuries,  in  contrast  to  aphasia,  which  appears  only  after  lesions 
on  the  left  side. 

2.  Contractures  and  Hypertonia 

A  further  factor  in  restricting  motion  (myogenic  contracture)  is  added  to 
actual  muscular  weakness  in  hemiplegia,  after  the  initial  flaccid  palsy  has 
passed  off. 

By  this  one  understands  the  fixation  of  limbs  in  a  definite  position  by  the 
occurrence  of  involuntary  permanent  shortening  of  muscles. 

These  myogenic  contractures  are  to  be  distinguished  from  the  contrac- 
tures due  to  cutaneous  scars,  ankylosis,  or  shortening  of  ligaments  or 
tendons. 

The  normal  tension  of  the  muscles,  fixes  in  a  certain  measure,  in  healthy 
conditions,  the  limbs  in  their  positions  at  all  times,  so  that  in  sudden  and 
quick  passive  movement  of  a  limb,  a  moderate  resistance  of  the  stretched 
muscle  is  felt.  This  normal  resistance  is  lacking  in  some  nervous  diseases, 
especially  in  tabes.  Then  we  speak  of  increased  passive  motility  or  of 
hypotonia. 


494  DISEASES  OF  THE  BRAIN 

On  the  contrary,  the  resistance  to  passive  movement,  in  diseases  of  the 
pyramidal  tracts,  is  very  much  increased :  hypertonia  of  certain  muscles  and, 
therefore,  fixation  in  definite  positions  appears. 

In  this  fixation,  there  is  a  second  factor;  frequently  there  appears  besides 
hypertonia,  connective-tissue  contraction.  Contraction  of  the  connective  tissue 
of  a  muscle  appears,  when  its  points  of  insertion  are  not  separated  from  one 
another  for  a  long  time,  therefore,  in  the  antagonists  of  a  paralyzed  muscle, 
even  if 'it  is  not  in  the  condition  of  hypertonia.  Shrinking  contracture,  there- 
fore, appears  also  in  flaccid  paralyses,  as,  for  instance,  in  poliomyelitis. 

In  spastic  -paralyses,  which  have  existed  for  a  long  time,  there  is  added 
this  shrinking  contracture  to  the  hypertonic  or  spastic  contractures. 

These  shrinking  contractures  must,  therefore,  be  differentiated  from  the 
spastic,  which  are  conditioned  not  by  tissue  changes,  but  by  nervous  hyper- 
tonia. This  hypertonia  exhibits  itself  in  hemiparetic  limbs,  even  when  it 
does  not  go  so  far  as  contracture,  by  increased  spring-like  resistance  on 
sudden  passive  flexion  or  extension,  and  corresponds  to  the  spasm,  which 
appears  with  the  patient's  own  movements.     The  reflexes  are  exaggerated. 

Sometimes  one  sees,  either  at  once  or  soon  after  a  cerebral  injury  occurs 
(hematoma  of  the  dura  or  trauma,  meningitis),  spasmodic,  rigid,  abnormal 
attitudes  and  positions  of  the  limbs,  which,  unfortunately,  have  been  called 
early  contractures.  They  usually  disappear  again  and  alternate  with  tonic- 
clonic  spasms.  They  are  conditions  of  irritation,  better  included  under  tonic 
spasms.  If  one  speaks  broadly  of  contractures,  one  does  not  mean  these 
transient  increases  of  tension  caused  by  special  irritation,  but  permanent 
conditions  of  contracture,  which  appear  as  a  permanent  symptom  in  from 
a  few  days  to  one  or  two  weeks,  after  apoplexy,  resulting  from  a  lesion  in  any 
part  of  the  entire  motor  cortico-spinal  system,  that  is,  from  the  cortex  of  the 
anterior  cerebral  convolution  to,  but  not  including,  the  anterior  cornu  cells. 

Unless  therapeutic  preventative  measures  are  taken  in  these  cerebro- 
spinal paralyses,  a  shrinking  contracture  in  the  course  of  time  is  added  to 
the  spastic  contracture. 

Spastic  contractures  are  relaxed  in  narcosis,  in  deep  sleep,  under  the  effect 
of  constriction  in  Esmarch's  bandaging  of  the  affected  limb,  and  also  partly 
in  a  warm  bath. 

Naturally  shrinking  contracture  can  not,  under  these  circumstances,  be 
relaxed,  and  thereby  the  part  that  hypotonia  and  shrinking  play  in  contrac- 
ture, can  easily  be  determined.  Mental  excitement  increases  spastic  con- 
tracture. Spastic  contracture  shows  a  spring-like  resistance  against  attempts 
to  cause  relaxation.  It  is  violent  in  repelling  sudden  brusque  attempts; 
on  the  other  hand,  many  contractures  can  be  easily  relaxed  by  gradual 
procedure.  Attempts  at  movement  both  in  the  affected  and  the  unaffected 
limbs,  increase  the  contracture. 

The  characteristic  positions  of  the  limbs  of  patients  suffering  from  mono- 


CONTRACTURES  495 

hemi-,  and  diplegia,  appear  only  because  of  differences  in  the  degree  of 
contracture  in  the  various  groups  of  muscles. 

There  is,  indeed,  absolutely  no  regular  form  of  hemiplegic  contracture, 
but  one  type  is  the  most  frequent.  On  the  whole,  we  find  contracture  in  those 
muscles  that  are  relatively  free  from  paralysis  (cf.  page  492),  that  is,  the 
antagonists  of  the  "predilection"  muscles.  The  typical  position  of  the 
hemiplegic  arm  corresponds,  therefore,  to  the  power  retained  by  the  flexors 
of  the  hand,  the  inner  rotators  and  flexors  of  the  arm :  the  fingers  are  closed, 
the  thumb  turned  in  or  adducted,  the  arm  lies  close  to  the  breast,  bent  at 
the  elbow,  the  leg  in  extension,  the  foot  in  the  position  of  equino-varus. 
Because  of  the  lengthening  of  the  leg  by  the  plantar  contracture  or  weakness 
of  dorsal  flexion,  the  leg  in  walking  is  moved  in  a  circle  (circumducted). 
This  is  the  usual  distribution  of  contractures,  in  which  generally,  the  muscles 
that  may  still  be  somewhat  moved  at  will,  are  contracted.  Sometimes, 
but  more  seldom  the  opposite  takes  place:  flexion  and  contraction  of  the 
leg,  extension  and  contraction  of  the  arm  and  fingers. 

The  distribution  of  contractures  and  the  corresponding  permanent  posi- 
tion of  the  paralyzed  limbs,  certainly  depend  on  the  fact  that  certain  muscles 
are  relatively  spared.  But  only  indirectly,  through  the  fact,  that  the  return 
of  a  certain  power  in  single  groups  of  muscles  is  decisive  for  the  position  of 
the  limb  in  the  first  weeks  after  the  paralysis  occurs.  In  the  final  issue,  the 
position  assumed  by  the  limb  during  the  time  of  relaxed  and  semi-relaxed 
paralysis  determines  the  distribution  of  contractures.  Each  group  of 
muscles  is  inclined  in  pyramidal  disease  to  adapt  itself  to  an  approach  of  the 
insertion  points,  brought  about  by  any  cause  whatsoever,  through  in- 
crease in  tension  and  shortening.  This  is  a  subcortical  reflex,  which  ap- 
pears only  after  the  "isolation"  of  the  subcortical  centers  (and  besides  de- 
pends on  the  intactness  of  centripetal  pathways,  hence  is  missing  when 
tabes  exists). 

That  this  assumption  of  the  dependence  of  contractures  on  the  position 
taken  by  the  limb  after  paralysis,  is  correct,  is  proved  by  the  fact  that 
one  is  able,  by  artificial  fixation  of  a  limb,  to  change  and  transform  an 
already  existing  flexion  contracture  into  an  extension  contracture  and 
vice  versa. 

The  return  of  power  to  certain  groups  of  muscles  (the  antagonists  of  the 
preferred  (predilection)  muscles)  seems,  therefore,  to  lead  to  contracture 
only  by  causing  the  choice  of  a  certain  position  of  the  limbs  in  the  first  weeks 
after  the  paralysis.  Hence  it  happens  that,  if,  by  external  conditions,  the 
limb  is  prevented  from  following  this  influence,  the  distribution  of  contrac- 
ture is  changed,  and  in  this  way  mainly  can  we  account  for  the  various 
modifications  of  the  above  described  typical  position  of  the  limbs  in 
hemiplegia. 


496 


DISEASES  OF  THE  BRAIN 


(3)  Differences  in  Cerebral  Paralysis  According  to  the  Situation  of  the  Lesion 

(a)  Cortical  Focal  Lesions  " 

The  fact  that  the  motor  paths  in  the  inner  capsule  and  the  cerebral 
peduncle  are  placed  closely  side  by  side,  on  the  one  hand,  and  the  fact  that 


Sensory  tract-  — —  \  — 


Fig.  134. — The  principal  segments  of  the  internal  capsule.  Bkn,  Genu  of  the  corpus  callosum; 
Vh,  anterior  horn;  Knee  ic,  genu  of  the  inner  capsule;  /  optic,  lenticulo-optical  section  of  the 
internal  capsule;  CA,  cornu  ammoni;  Hh,  posterior  horn;  calc,  fissura  calcarina;  .F3,  third  frontal 
convolution;  sta,  corona  radiata;  I  sir  ic,  lenticulo-striate  section  of  the  internal  capsule;  c  ext, 
capsula  externa;  Z,  island  of  Reil;  Operc,  operculum;  CI,  claustrum;  Ti,  first  temporal  convolu- 
tion; r  lie,  retro-lenticular  section  of  the  internal  capsule;  Ss,  optic  radiations;  T2,  second  temporal 
convolution.     (After  v.  Monakow.) 

they  are  separated  in  the  white  matter,  corresponding  to  the  various  parts 
of  the  anterior  central  convolution,  from  which  they  arise,  on  the  other  hand, 


CORTICAL  FOCAL  LESIONS 


497 


cause  a  difference  in  the  effects  of  focal  injuries,  that  affect  the  cortex  and 

parts  of  the  adjacent  white  matter  from  those  focal  injuries  affecting  the 

inner  capsule,  or  the  cerebral 

peduncle,  and,  of  course,  the 

still   deeper  lying  planes  of 

the  motor  path.     The  paths 

for  the  leg   come   from  the 

paracentral  lobule  and   the 

upper  fourth  of  the  anterior 

central    convolution,    those 

for  the  arm  and  hand  from 

the      middle      two-fourths, 

those  for  the   face,  tongue, 

masticatory    muscles,    from 

the  lower  fourth  of  the  same 

convolution. 

Focal  injuries  in  or  near 
the  cortex,  of  moderate  ex- 
tent, paralyze  only  arm  or 
leg  or  face,  that  is,  cause  so- 
called  monoplegias,  whereas 
focal  injuries  more  deeply 
situated  paralyze  most  fre- 
quently the  entire  contralat- 
eral side,  causing  hemi- 
plegia. 

Monoplegia  of  the  face  is 
often  combined  with  that  of 
the  arm:  monoplegia  facio- 
linguo-brachialis;  that  of  the 
leg,  often  with  that  of  the 
arm,  monoplegia  brachio- 
cruralis. 


Ant. pyramid  Col. 


Fig.  135. — Scheme  for  the  explanation  of  hemiplegia 
alternans   (for   the   seventh   and   twelfth  nerves).      Red: 
pyramidal    tract.    N  .VIIs,    Nerv.    facialis    sinister;  N. 
A   lesion    entirely   in    the   XIId>   right   hypoglossal   nerve.     I,   Focus  in   the  right 

internal  capsule,  left  half  of  the  body,  left  seventh  and 
paracentral  lobule  Causes  twelfth  nerves  affected;  II,  in  the  right  peduncle,  the 
merelv    -bnrnU^^    nf  ih?   Ua     same;  HI>  focus  m  the  rfght  half  of  the  pons,  left  half  of 

merely  paralysis  oj  me  leg.  me  body  and  left  twelfth  nerVG)  right  sev£nth'nerve  (sup_ 

As  the  paracentral  lobule  is   ranuclearly),  affected;  IV,  in  the  same  place  caudad,  left 

,.    ,    ,  .  half  of  the  body,  left  half  of  the  tongue  and  right  seventh 

Supplied    by    another   artery   nerve  affected  (nuclearly  resp.  radicularly);  V,  focus  in  the 

than  the  central  convolution,  Z^\lTlYZts^\  M\  haH  °ith?  W  and  the  right 

»    nali  of  the  tongue  (nuclearly  or  radicularly) . 

namely  the  corpus  callosum 

artery,  this  isolated  paralysis,  combined  with  softening  of  the  corpus  callosum 

occurs  when  the  above-named  artery  is  occluded. 


32 


498  DISEASES  OF  THE  BRAIN 

Therefore  the  characteristics  of  cortical  focal  injuries,  or  of  focal  injuries 
lying  near  the  cortex  are : 

i.  The  paralysis  is  confined  to  one  or  two  members  (arm,  leg,  face). 

2.  Localized  Jacksonian  convulsions. 

In  lesions  of  the  central  convolutions,  inner  capsule,  etc.,  the  muscles 
of  the  eye  are  not  affected.     For  conjugate  deviation  see  page  478.    . 

(/?)  Capsular  Focal  Lesions 

In  the  internal  capsule,  all  the  motor  paths  are  easily  stricken  together; 
they  lie  back  of  the  so-called  knee  in  the  front  third  of  the  posterior  limb. 

The  order  of  fibre  bundles,  from  the  knee  backwards,  is  as  follows: 

Facial  fibres,  tongue  fibres,  arm  fibres,  leg  fibres  (Fig.  134).  The  fibres 
are  so  indistinctly  separated  that  a  monoplegic  paralysis  through  capsular 
focal  injury  is  exceedingly  rare.  It  has  been  observed  that  the  leg  is  mostly 
affected  by  an  injury  situated  in  the  hind  part,  the  face  by  one  in  the  front. 

Focal  injuries  in  the  cortex  and  the  white  matter  differ  in  one  respect, 
that  has  long  been  disregarded,  from  those  in  the  inner  capsule  and  still 
deeper  lying  parts  of  the  pyramidal  tract.  The  former  affect  the  fibrillation 
of  the  hemisphere,  where  it  contains  closely  mixed,  projection  fibres  and 
corpus  callosum  fibres,  destroy  therefore  besides  these,  countless  connections 
with  the  other  hemisphere.  On  the  contrary,  focal  injuries  in  the  inner 
capsule,  in  the  peduncle,  etc.,  destroy  no  commissural  fibres  and  therefore 
leave  the  harmonious  co-operation  of  the  two  hemispheres  undisturbed.  A 
consequence  of  this  difference  we  shall  learn  later  on  under  apraxia. 

(f)  Focal  Lesions  in  the  Peduncle  and  Pons.    A  Iternate  Paralyses 

If  a  focal  injury  affects  the  motor  path  in  the  peduncle,  where  it  is  situated 
in  the  middle  third  of  its  foot,  the  result  for  the  extremities  is  the  same  as  if 
there  were  a  break  in  the  inner  capsule. 

But  for  the  cerebral  nerves  here  and  in  the  pons,  certain  peculiarities 
occur.  Whereas  the  pyramidal  tracts  for  the  extremities  do  not  cross  until 
the  medulla  is  reached,  the  corresponding  paths  for  the  cerebral  nerves 
pass  already  in  the  peduncular  layers  or  pontile  layers  to  the  nuclei  of  the 
nerves  concerned  on  the  other  side  (third,  fourth,  fifth,  sixth  and  seventh 
nerves).  Such  a  focal  injury  in  the  left  peduncle  may  strike  the  entire 
cortico-spinal  path  for  the  right  extremities,  and  also  the  nucleus  or  root 
of  the  left  oculomotor  nerve.  Consequently  to  the  spastic  paralysis  of  the 
right  limbs  and  the  right  seventh  nerve,  is  added  a  nuclear  or  peripheral 
paralysis  of  the  left  oculomotor  nerve  (ptosis,  internus,  paralysis,  etc.). 
This  is  called  hemiplegia  alternans  (alternate  hemiplegia) . 

The  same  thing,  moreover,  can  take  place  in  the  pons  for  the  trigeminus, 
abducens,  and  facial  nerves.  Then  to  the  paralysis  of  the  limbs  of  one  side 
is  added  paralysis  of  the  trigeminus,  abducens  or  facial  nerve,  on  the  other 


CORTICAL  FOCAL  LESIONS,     VOLUME  OF  MUSCLES 


499 


side,  and  moreover,  when  nucleus  or  root  is  affected,  the  paralysis  is  of  a 
peripheral  character.  It  may  happen,  however,  that  only  the  supranuclear 
bundles  of  the  central  neuron  are  affected. 

Because  of  the  relation  of  the  nuclei  to  the  pyramidal  tracts,  many 
combinations  are  possible.  For  instance,  when  the  focal  injury  crosses  the 
middle  line,  both  seventh  nerves  or  both  twelfth  nerves  may  be  stricken  by 
supranuclear  paralysis,  or  one  by  supranuclear  and  the  other  by  -tmclear 
paralysis  besides  a  hemiplegia  (Fig.  135). 


Ftllet 


Red  nucleus  "m-       1  "^w*aiB*e*^, 

Fig.  136. — Hemiplegia  alternans  superior.  The  focus  H  interrupts  the  right  pyramidal 
tract  2  and  the  right  oculomotor  root  N.III  and  produces  therefore  paralysis  of  the  left  extremities 
and  of  the  right  eye. 

The  combination  of  hemiplegia  with  crossed  oculomotor  paralysis 
(peduncular  focal  injury)  is  known  as  Weber's  paralysis  or  hemiplegia 
alternans  superior. 

Hemiplegia  with  crossed  paralysis  of  the  facial  or  abducens  nerve  must 
result  from  a  focal  injury  in  the  pons  (Gubler's  paralysis).  Hemiplegia 
alternans  inferior. 

In  the  medulla,  a  focal  injury  may  strike  the  as  yet  uncrossed  pyramidal 
tract  of  the  one  side,  and  the  root  of  the  hypoglossal  nerve  of  the  same  side; 
then  the  limbs  are  spastically  paralyzed  on  one  side  and  the  tongue  atrophic- 
ally  paralyzed  on  the  other  (hemiplegia  alternans  infima). 

Just  as  cerebral  motor  nerves  crossed  to  the  extremities,  can  be  paralyzed 
by  focal  injuries  in  the  peduncle  and  pons,  there  can  be  disturbances  in  the 
crossed  sensory  nerves,  of  such  kind,  that  with  the  pyramidal  tract  of  the  right 
half  of  the  pons,  the  right  root  of  the  trigeminal  nerve  is  affected,  hence 
with  paralysis  of  the  left  side  of  the  body,  disturbances  in  sensibility  in  the 
right  side  of  the  face  may  appear  (Fig.  137). 

These  interchanging  paralyses  of  the  cerebral  nerves  are  important  local 
symptoms  of  lesions  in  the  peduncle  and  pons. 

Volume  of  Muscles 

In  contradistinction  to  nuclear  and  peripheral  paralyses,  the  muscles 
in  cerebral  paralysis  retain,  at  first,  their  volume  and  gradually  atrophy  at  a 


5oo 


DISEASES  OF  THE  BRAIN 


rate  corresponding  to  their  remaining  unused.  Atrophy  is  not  so  marked 
as  in  nuclear  and  peripheral  palsy.  Likewise  electric  reaction  is  not  quali- 
tatively changed  in  muscles  and  nerves,  and  quantitatively  is  but  slightly 
lowered.  Exceptions  are  rare  (atrophy  and  electric  changes).  Their 
cause  is  not  known. 

Vasomotor  and  trophic  disturbances  are  observed  in  hemiplegic  and 
sometimes  in  merely  hemiansesthetic  limbs;  changes  in  temperature,  cyano- 
sis, edema,  joint  diseases,  skin  diseases,  etc.,  also  occur. 


NIV.  HL 


N.IV 
Aq 


LSchl 


Vw 


Fig.  137. — Relation  of  the  trigeminal  to  the  pyramidal  tract  in  the  pons.  A  focus,  which 
strikes  the  root  of  the  fifth  nerve  and  the  pyramidal  tract  Py,  produces  synchronous  paralysis  of 
sensibility  in  the  face  and  crossed  hemiplegia.  Sch.m  and  Sch.l.,  middle  and  lateral  parts  of  the 
principal  fillet;  L.Schl,  lateral  or  lower  fillet;  fr,  forma  no  reticularis;  BA,  superior  peduncle;  HL, 
posterior  longitudinal  bundle;  N.IV,  trochlearis,  Vw,  root  of  the  trigeminal.     (After  v.  Monakow.) 

Bilateral  focal  injuries  cause  bilateral  hemiplegia — diplegia.  Focal 
injuries  that  strike  the  paracentral  lobules  only,  paralyze  both  legs  (cerebral 
paraplegia) . 

(d)  Sensory  Symptoms  of  Lost  Function 

General  sensation  includes  various  qualities : 

1.  Tactile  sensations.1 

2.  Sensations  of  pain   (loss:  analgesia  or 
hypalgesia) . 

3.  Thermic    sensations    (loss:   thermoan- 
sesthesia). 

4.  Sensations  of  place  (localization  of  the 
place  of  stimulation). 

1  Striimpell  differentiates  pressure  sensations  of  the  deeper  parts. 


Superficial  sensibility. 


SENSORY  SYMPTOMS  OF  LOST  FUNCTION  501 

Sensations  of  space.  Differentiation  of 
two  simultaneously  stimulated  points  on 
the  skin  as  separate  (Weber's  circles  of 
touch) . 


6.  Sensations  of  situation  and  of  movement, 
the  latter  both  passive  and  active. 

7.  Sensations  of  weight  and  resistance. 


Deep  sensibility  of 
joints,  tendons,  mus- 
cles (also  called  mus- 
cular sense). 


A  "  stereognostic "  sense,  that  is  the  ability  to  recognize  the  form  of  a 
body  from  sensations  of  touch,  position,  movement,  obtained  by  handling  it, 
may  not  be  placed  with  the  elementary  qualities. 

Aster eo gnosis,  in  so  far  as  it  is  not  the  consequence  of  the  loss  of  ele- 
mentary sensations,  belongs  among  agnostic  symptoms.     (See  page  541.) 

A  loss  of  sensation  may  be  partial,  in  so  far  as  only  certain  qualities  are 
lost,  for  instance  only  sensations  of  position  and  movement  {dissociation  of 
sensibility) . 

It  can  also  be  partial  in  relation  to  its  intensity  (loss,  mere  diminution  of 
sensibility) . 

Finally  in  relation  to  its  extent.  Only  part  of  half  of  the  body,  or  only 
part  of  a  member  is  anaesthetic  or  hypaesthetic. 

In  order  to  cause  complete  loss  of  all  sensation  in  the  distribution  of  any 
nerve,  either  the  nerve  or  its  root  must  be  completely  cut  off.  Even  then 
partial  compensation  may  occur  in  the  course  of  time  because  of  the  partial 
overlapping  of  the  sensory  areas  by  various  roots. 

Such  total  anaesthesia  can  be  caused  by  cerebral  focal  injuries  only  in  the 
pons  or  medulla  oblongata,  since  only  in  these  places  can  the  peripheral 
neuron  of  a  nerve  which  subserves  general  sensibility,  namely,  the  root  of  the 
trigeminus  be  affected.  More  centrally,  a  supranuclear  central  neuron  is 
always  struck  by  the  focal  injury.  Lesions  of  the  central  sensory  pathways, 
in  whatever  plane  they  may  lie,  usually  do  not  totally  destroy  sensibility  in  any 
of  the  three  directions  (quality,  intensity,  extent). 

Anaesthesias  of  the  entire  half  of  the  body,  contralateral  to  the  injury, 
endure  only  for  the  first  days  or  at  most  weeks  after  the  attack. 

In  particular,  sensibility  to  pain  is  nearly  always  to,  a  certain  extent 
restored,  even  if  it  frequently  remain  diminished. 

Next  thermic  sensibility  is  most  frequently  restored,  and  after  it  sensibility 
to  touch.  Sensations  of  place  and  space,  position  and  movement,  on  the  con- 
trary, in  cases  of  central  focal  injuries,  are  often  permanently  destroyed. 

On  the  whole,  even  in  large  cerebral  focal  injuries,  sensory  processes  are 
better  restored  than  motor  processes. 

Concerning  the  extent  of  sensorial  disturbances,  it  must  be  noticed,  that  at 


502  DISEASES  OF  THE  BRAIN 

first  they  frequently  exhibit  an  hemianaesthetic  character  (the  anaesthesia 
ceases  precisely  at  the  middle  line)  but  that  later  the  boundary  recedes  from 
the  middle  line — the  medial  parts  of  the  head  and  neck  again  become  sensi- 
tive— and  the  disturbance  becomes  less  from  the  distal  parts  of  the  body, 
hand,  foot,  etc. 

Hemianaesthesia,  as  long  as  it  is  total,  reaches  the  middle  line  and  affects 
not  only  the  skin,  but  the  mucous  membranes,  joints  and  muscles.  It  is 
accompanied  usually  by  hemiplegia,  but  there  are  also  cases  of  isolated  and 
nearly  isolated  cerebral  sensory  disturbances. 

Anaesthesia  may  also  confine  itself  wholly  or  approximately  to  single 
limbs  or  parts  of  limbs.  (In  that  case,  the  distal  parts  are  more  seriously 
affected.) 

Generally  the  more  seriously  paralyzed  limb,  is  also  more  seriously 
impaired  in   respect  to  its  sensory   functions. 

The  dissociation  of  sensibility  by  cerebral  lesions  may  in  exceptional 
cases  go  so  far,  that  cold  sensations  are  retained,  while  heat  sensations  are 
lost. 

Concerning  ataxia  of  the  injured  side,  in  so  far  as  it  is  connected  with 
disturbances  in  muscular  sense,  see  next  division. 

The  above  general  statements  concerning  the  usual  incomplete  loss  of 
sensibility,  or,  at  least,  the  probable  restoration  of  part  of  the  sensibility,  holds 
good  first  of  all,  for  the  cortical  parts  and  those  lying  near  the  cortex,  but  to  a 
certain  degree  also  for  focal  injuries  in  the  thalamus,  even  for  those  in  the 
pons  and  medulla. 

The  sensory  pathways  obviously  have  manifold  lateral  branches — as  is 
proved  by  their  frequent  interruption  by  gray  matter.  One  must  consider 
even  the  vicarious  entrance  of  the  other  hemisphere,  since  in  some  cases 
return  of  a  considerable  amount  of  sensibility  has  been  observed,  though  all 
the  sensory  paths  of  one  hemisphere  had  been  totally  destroyed.  Gradual 
processes,  like  tumors,  cause  far  less  sensorial  disturbances,  than  those 
appearing  suddenly. 

Peculiarities  of  focal  injuries  at  various  levels  must  be  discussed  under  a 
review  of  the  sensory  paths. 

Our  knowledge  of  the  sensory  pathways  is,  as  yet,  very  incomplete; 
opinions  vary  greatly  on  the  more  delicate  relations  of  parts;  therefore  the 
following  statement  can  give  only  a  rough  schematic  sketch. 

The  centripetal  paths,  that  course  to  the  cerebellum,  and  do  not  cause 
stimulation  that  leads  to  conscious  perception  (lateral  cerebellar  tract  and 
Gower's  tract)  have  been  mentioned  in  the  discussion  of  the  cerebellum  (page 
480)  and  are  passed  by  here. 

The  major  part  of  the  sensory  tracts,  which,  uncrossed,  run  through  the 
posterior  columns  of  the  spinal  cord  (paths  for  the  muscular  sense)  and  end  in 
the  posterior  column  nuclei  (spino-bulbar  tract)  takes  from  these  its  continua- 


THE  SENSORY  PATHWA  YS 


5o3 


tion  in  the  central  (bulbothalamic)  sensory  path,  which,  above  the  pyramidal 
decussation,  also  crosses  as  fibrse  arcuatas  internae  (superior  pyramidal  decus- 
sation) and  forms  the  inter-olivary  layer  (see  Figs.  123  and  124).  These 
fibres,  after  crossing  reach  the  medial  or  principal  fillet  (Fig.  138),  which  ends 
in  the  optic  thalamus.  For  the  situation  of  the  main  fillet  in  the  pons  see 
Fig.  137. 

That  part  of  the  sensory  fibres  that  has  crossed  in  the  spinal  cord 
(tract,  spino-thalamicus) ,  and  passes  through  the  lateral  tract  (pain  and 
temperature  conductors),    consisting  possibly  of  a  chain  of  short  tracts, 


Fig.  138. — General  course  of  the  medial  or  principal  fillet.     (After  Edinger.) 

reaches  the  reticular  formation  of  the  pons  and  joins  the  principal  fillet,  in 
order  to  reach  the  optic  thalamus  together  with  it. 

Touch  sensations  seem  to  pass  through  both,  posterior  column  and  lateral 
tract. 

The  separate  position  of  the  paths  for  the  muscular  sense  on  the  one  hand 
(inter-olivary  layer,  fillet),  for  pain  and  thermic  sensations,  on  the  other 
(reticular  formation — subst.  reticularis),  in  the  medulla  and  the  more  caudal 
planes  of  the  pons,  makes  it  possible,  that  a  focal  injury  here  may  cause  such 
a  dissociation  of  sensibility  that  position  and  movement  sensations  are  re- 
tained, and  pain  and  thermic  sensations  lost,  or  vice  versa;  since  the  paths 
for  the  muscular  sense  cross  only  in  the  medulla,  it  depends  on  the  plane,  in 


5°4 


DISEASES  OF  THE  BRAIN 


which  the  focal  injury  lies,  whether  the  sensory  disturbance  is  on  the  crossed 
or  uncrossed  side.  The  fact  that  the  paths  of  the  lateral  tract  have  already 
crossed  in  the  spinal  cord,  makes  possible  very  complicated  results  (as  loss 
of  muscular  sense  on  the  same  side,  and  of  the  thermic  and  pain  sensations 
on  the  opposite  side). 

Focal  injuries  in  the  pons,  which  strike  the  trigeminus  roots  or  nuclei 
and  the  already  crossed  sensory  paths,  cause  sensorial  disturbances  in  the 
face,  on  the  same  side  as  the  injury,  and  in  the  body,  on  the  opposite  side 
(hemianesthesia  cruciata)   (Fig.  140). 

The  central  path  that  rises  in  the  nuclei  of  the  sensory  cerebral  nerves 
(fifth  nerve,  etc.),  and  probably  also,  as  has  been  said,  on  proximal  planes 
of  the  pons,  the  central  continuation  of  the  lateral  tract,  that  has  reached  the 


Ant.  Quad,  bodies 


Right 


Fig.  139. — Foci  in  the  quadrigeminal  region.  Focus  1  produces  hemianopia  (at  the  right 
side).  Focus  2  diminishes  the  auditory  acumen  in  both  ears.  If  combined  with  a  lesion  of 
the  auditory  tracts  at  the  right  side  it  produces  bilateral  deafness.  Focus  3  produces  disturbance 
in  sensibility  at  the  right  side  (because  of  the  lesion  of  the  fillet)  and  paralysis  of  the  oculomotor 
at  the  left  side.  Focus  4  produces  disturbance  of  motility  in  the  right  arm  and  leg  and  lesion 
of  the  oculomotor  at  the  left  side  (hemiplegia  alternans  superior) . 

reticular  formation,  and  is  there  interrupted  by  gray  matter,  join  the  prin- 
cipal fillet.     Thus  all  the  sensory  paths  reach  the  thalamus. 

On  the  entire  way  to  the  thalamus,  the  sensory  paths  are  separated  from 
the  motor  paths  and  can,  therefore,  obviously  be  interrupted  by  injuries  that 
spare  the  motor  paths.  Thus,  for  instance  in  the  frontal  division,  through- 
out the  hindmost  part  of  the  thalamus,  the  sensory  paths  lie  in  a  position 
clearly  dorsal  (in  the  tegmental  region)  from  the  pyramidal  paths  that  go 
through  the  foot  of  the  cerebral  peduncle. 

From  the  thalamus,  the  thalamo-cortical  path  passes  through  the  posterior 
limb  of  the  inner  capsule,  medially  from  the  lenticular  nucleus,  and  partly 
through  it  to  the  cortex. 

The  former  supposition  that  the  sensory  paths  in  the  posterior  limb  of 
the  inner  capsule  are  placed  absolutely  separate  from  the  motor  paths,  and 
lie  behind  the  latter  (Fig.  134),  has  been  impossible  to  maintain. 


THE  SENSORY  PA  THWA  Y  5o5 

The  isolated  sensorial  losses  often  noticed  in  focal  injuries  situated  here 
(with  motility  intact)  are  caused,  in  the  majority  of  cases  by  the  participation 
of  the  optic  thalamus  in  the  focal  injury.  But  even  if  the  sensory  paths  of 
the  inner  capsule  run  partly  together  with  the  motor  paths  it  is  still  possible 
for  them  to  be  struck  comparatively  isolated  and  en  masse,  in  the  place, 
where  they  pass  from  the  ventral  optic  thalamus  nucleus  into  the  posterior 
limb  of  the  inner  capsule,  so  that  focal  injuries  in  the  hind  third  of  the 
posterior  limb  of  the  inner  capsule  cause  more  damage  to  sensibility  than  to 
motility. 

Because  of  the  proximity  of  the  central  visual  path  passing  through  the 
hindmost  part  of  the  inner  capsule,  the  capsular  sensory  disturbances  are 
generally  combined  with  hemianopia. 

The  ends  of  the  sensory  paths,  lie  mainly  and  most  densely  in  the  posterior 
central  convolution.  The  anterior  one  receives  only  few  sensory  fibres. 
But  besides  the  posterior  central  convolution,  the  anterior  division  of  the  pari- 
etal lobe  receives  them. 


Superior 
peduncle 


Fig.  140. — Effect  of  a  focus  in  the  middle  of  the  pons.  (Exit  of  the  trigeminal  nerve.)  A, 
Aqueduct;  B,  pyramidal  tract.  Focus  1,  striking  only  the  fillet,  produces  crossed  disturbance 
of  sensibility  in  the  body.  Focus  2,  striking  besides  the  root  of  the  trigeminal,  produces  dis- 
turbance insensibility  of  the  face  on  the  same  side,  crossed  disturbance  of  sensibility  in  the  body 
(Hemianaesthesia  cruciata).    Focus  3  produces  crossed  motor  and  sensory  disturbance. 

Focal  injuries  in  the  posterior  central  convolution,  affect  with  special  force, 
sensations  of  place,  position  and  movement. 

Localization  by  the  patient,  in  that  case,  is  extremely  poor,  and  the 
consciousness  of  the  position  of  the  limb  is  often  wholly  lost.  On  the  con- 
trary, the  other  qualities,  pain,  temperature,  and  touch  sensations,  are 
attached  to  extensive  cortical  fields  (both  central  convolutions)  and  other 
regions,  especially  of  the  upper  parietal  lobe,  and  are  affected  more  or  less 
slightly  in  mere  focal  injuries  of  the  posterior  central  convolution.  Great 
destruction  of  the  posterior  central  convolution,  besides  that  of  the  adjacent 
parts  of  the  parietal  lobe  can  cause  serious  hemianassthesia,  which,  however, 
as  has  been  said  before,  may  retrogress  to  a  considerable  degree,  in  the  course 
of  time,  except  that  the  loss  of  muscular  sense  is  more  permanent.     (Some 


5o6  DISEASES  OF  THE  BRAIN 

authorities  hold  that  the  anterior  central  conventions  have  no  sensory- 
functions.) 

When  sensations  of  place,  position  and  movement  are  lost,  and  those  of 
touch  injured,  naturally  the  forms  of  objects  are  not  recognized,  by  feeling 
them,  and  the  objects  themselves  can  not  be  identified.  This  is  a  perceptive 
astereognosia  which  is  a  natural  consequence  of  the  loss  of  the  elementary 
qualities  needed  for  recognition  of  form,  and  must  not  be  confused  with 
Wernicke  touch-crippling  (touch-paralysis,  tactile  agnosia).  It  is  to  the 
latter  what  the  blind  man's  inability  to  read  is  to  alexia. 

The  above  mentioned  tactile  agnosia,  appearing  in  lesions  of  the  middle 
third  of  the  posterior  central  convolution  and  its  immediate  vicinity  towards 
the  rear,  cortical  touch-paralysis,  is  discussed  hereafter. 

Figs.  139  and  140  illustrate  the  effects  of  variously  situated  focal  injuries 
in  the  quadrigeminal  region  and  the  pons. 

(e)  Cerebral  and  Cerebellar  Ataxia 

As  destruction  of  the  posterior  column  of  the  spinal  cord,  through  the 
loss  of  centripetal  signals,  which  regulate  movement,  especially  of  the 
stimuli  coming  from  the  muscles,  joints,  ligaments,  causes  spinal  ataxia,  so 
extensive  breaks  in  these  sensory  paths,  in  the  higher  divisions — in  the 
medulla,  in  the  cap  of  the  pons,  in  the  cerebral  peduncle,  in  the  thalamus,  in 
the  inner  capsule,  centrum  semiovale  and  cortex — cause  cerebral  ataxia. 
Hence,  especially,  lesions  of  the  fillet,  optic  thalamus,  thalamo-cortical  teg- 
mental path,  the  cortex  of  the  posterior  central  convolution  and  of  the 
adjacent  parietal  cortex,  disturb  the  co-ordination  of  movements. 

Cortical  ataxia  and  subcortical  ataxia,  which  is  caused  by  a  break  in  the 
sensory  paths  and  is  with  difficulty  differentiated  from  it  present  a  somewhat 
different  picture  from  that  of  the  well  known  spinal  ataxia  of  tabes.  The 
excessive,  flail-like  movements  of  the  tabetic  are  not  so  prominent.  Instead, 
all  movements  become  inexact,  without  consideration  as  to  the  shortest  way; 
now  the  movement  comes  near  the  goal,  now  it  falls  short  of  it. 

The  fine  harmony  in  the  co-operation  of  muscles  is  absent;  neither  choice, 
nor  measure,  nor  sequence  in  time  of  the  innervations  is  correct.  The 
synergies  (co-operation  of  muscles  (agonists)  and  their  antagonists  and  of 
collateral  and  rotatory  synergists)  are  disordered.  Often  the  object  falls 
from  the  hand,  the  sensory  disturbance  directly  co-operating  with  ill  managed 
muscular  action.  The  writing  of  the  ataxic  is  scribbling,  shows  evidences  of 
trembling  and  does  not  stay  on  the  line;  the  letters  and  parts  of  letters  are 
uneven  and  contorted.  All  the  assurance,  exactness  and  finish,  that  practice 
has  given  our  movements,  are  lost.  In  what  was  formerly  called  cortical 
ataxia,  there  was  included,  besides  this  awkwardness,  which  is  conditioned 
by  the  loss  of  centripetal  control,  something,  now  classed  with   apraxia: 


DISORDERS  OF  THE  SPECIAL  SENSES  507 

kinetic  memories  for  certain  innervation  complexes  are  lost,  cf.  apraxia. 
(Probably  mimic  disturbances,  at  times  observed  in  focal  injuries  of  the  thala- 
mus are  merely  the  consequence  of  loss  in  sensibility.) 

Different  from  cortical  and  subcortical  cerebral  ataxia  (focal  injuries  in 
the  medulla,  pons,  etc.,  belong  here,  in  as  much,  as  they  act  through  lesions 
which  affect  paths  going  to  the  cerebrum)  is  cerebellar  ataxia.  The  cere- 
bellum has  less  to  do  with  the  co-ordination  of  the  movements  of  the  extremi- 
ties, than  with  the  regulation  of  upright  position,  of  the  maintenance  of  equi- 
librium in  standing  and  walking.  The  sufferer  from  cerebellar  disease  totters 
like  a  drunken  man.  The  characteristic  of  cerebellar  ataxia  is,  that  a  person 
exhibiting  it  in  its  severer  forms  when  walking  or  standing,  shows  compara- 
tively little  ataxia  in  arm  and  leg  movements  made  while  lying  on  his  back. 

Especially  in  walking  interference  is  shown  in  the  regular  co-operation 
of  legs  and  trunk.  This  may  be  only  unilateral  and  then  the  ataxia  is  on  the 
same  side  as  the  cerebellar  focal  injury  (in  contradistinction  to  cerebral 
focal  injuries).  This  control  by  the  cerebellum  occurs  probably  because  of 
the  stimulations  flowing  towards  it  through  the  lateral  cerebellar  tract,  which 
do  not  become  conscious,  whereas  the  usual  control  of  the  movements  of  the 
extremities  comes  from  the  cerebrum  and  is  based  for  the  most  part  on 
conscious  perceptions. 

/.  Symptoms   of  Lost  Sensory  Function  (Disturbances  of  the 

Special  Senses) 

1.  Visual  Disturbances,  Visual  Paths 

(a)  Choked  disc  has  been  discussed  under  general  symptoms.  See 
page  487. 

In  long  persisting  choked  disc,  central  keenness  of  vision  is  generally 
impaired,  and  the  field  of  vision  irregularly  restricted.  The  final  result  may 
be  blindness,  which  corresponds  ophthalmoscopically  to  the  clinical  picture 
of  atrophy  of  the  optic  nerve.  If  an  operation  be  had  in  time  or  perhaps  in 
case  of  cerebral  syphilis,  therapeutical  methods  be  used,  choked  disc  and 
optic  neuritis  may  disappear  entirely.  [It  should  never  be  forgotten  that 
a  quite  marked  optic  neuritis  may  be  present  and  the  patient  not  realize  any 
deficiency  of  vision.] 

(b)  Atrophy  of  the  optic  nerve,  greenish  whitish  discoloration  of  the 
well  defined  disc,  usually  bilateral,  is  a  rather  frequent  symptom  of  degenera- 
tive processes  in  tabes  and  tabo-paralysis,  occurring  less  often  in  pure  syph- 
ilis. It  often  precedes  the  other  symptoms  by  years.  One  must  be  very 
cautious,  therefore,  in  assuming  the  existence  of  isolated  optic  atrophy  without 
tabes,  paralysis,  or  syphilis.  It  may  happen,  however,  that  optic  atrophy 
exists  permanently  without  other  nervous  disease.  Optic  atrophy  of  slighter 
degree  is  found,  at  times,  in  multiple  sclerosis. 


5o8  DISEASES  OF  THE  BRAIN 

There  are,  besides,  other  bilateral  diseases  of  the  optic  nerve,  which 
cause  slight  or  no  changes  in  the  fundus  of  the  eye,  that  often  lead  to  sudden 
blindness  or  produce  only  central  scotoma  especially  for  red  and  green. 
They  are  traced  back  to  certain  neuritic  processes  in  the  optic  nerve 
(neuritis  retrobulbaris),  toxic  substances,  as  alcohol,  nicotine,  extract,  filic. 
mas.,  being  usually  the  cause.  A  more  detailed  description  of  them  belongs 
in  a  text-book  on  diseases  of  the  eye. 

Of  interest  is  "amaurotic  idiocy,"  recently  discovered,  a  combination  of 
optic  nerve  atrophy  with  peculiar  changes  in  the  macula  lutea  and  idiocy, 
which  in  certain  families  is  hereditary  and  found  chiefly  in  Jewish  children, 
(c)  Injuries  to  the  chiasm. 

Since  affections  that  attack  the  chiasm,  generally  proceed  from  the  middle, 
very  frequently  from  the  hypophysis,  it  frequently  happens  that  both  crossed 

bundles   of  the  optic  nerves — which  supply 

\v\  //■' /  ^e  nasal  retinal  surface — are  affected,  there - 

"\     \^^  -'  fore  the  temporal  halves  of  the  field  of  vision 

y'yy^Wd^S:  -^  are  blinded  (bitemporal  hemianopia) .     If  the 

/ ///      Herd      \V\  \         uncrossed  bundle  on  one  side  is  also  affected, 

/ ///  \vN      tota*  blindness  of  the  eye  on  that  side,  and  only 

'  T    .  ^N   '     temporal  blindness  of  the  other  results.     A 

Fig.  141. — Lesion  m  the  chiasm  .  ..  . 

arising  from  disease  in  the  hy-    lesion  arising  without  the  chiasm  that  affects 
Eet£opEd  ^"S^dTSi   it  (««»w  Fig.  141),  may  cause  unilateral  nasal 

are  indicated  by  dotted  lines.     The    hemianopia. 

crossed  fibres  affected  by  the  lesion  „,  ,  .  f    ,  .,  ,      , 

subservebothnasalhalvesoftheret-         The    combination    of   bitemporal    nemi- 

ina    therefore  the   temporal  halves    anopia    witn    acromegaly     IS    well  known   as 
of  the  -visual  field.  r  b     J 

characteristic  of  a  tumor  of  the  hypophysis. 

The  Visual  Paths. — Into  the  optic  tract  of  each  side,  enter  the  fibres  of 
the  same  sided  half  of  the  retina  of  that  side  and  the  (crossed)  fibres  of  the 
same  sided  half  of  the  retina  of  the  opposite  side.  As  to  their  main  body, 
the  tractus  fibres  end  in  the  lateral  geniculate  body;  a  smaller  part  ends  in  the 
anterior  quadrigeminal  body  and  in  the  pulvinar.  See  Fig.  142.  They  are 
the  so-called  primary  optical  centers.  The  fibres,  that  go  to  the  quadrigeminal 
body,  come  into  communication  with  the  oculomotor  nucleus,  and  produce 
the  pupillary  reflex. 

From  these  primary  centers  the  so-called  optic  radiation  passes  back  into 
the  occipital  lobe,  and,  surrounds,  in  its  entire  course  from  the  outside,  the 
posterior  horn.  In  this  course  it  passes  the  deep  white  matter  of  the  lower 
parietal  lobe  (especially  of  the  angular  gyrus)  and  occipital  lobe  and  the 
greater  part  of  it  reaches  the  medial  surface  of  the  occipital  lobe,  in  the  vicinity 
of  the  calcarine  fissure,  in  the  visual  center  marked  by  Vicq  d'Azyr's  stripe 
and  peculiar  formation.  This  embraces  mainly  the  cuneus  (dorsal  lip  of  the 
calcarina)  and  the  lobus  lingualis,  perhaps  also  the  fusiform  gyrus.     The 


THE  VISUAL  PATHS 


5°9 


visual  center  encroaches  but  little  upon  the  convexity,  except  perhaps  at  the 
occipital  pole  and  in  the  third  occipital  convolution. 

From  the  visual  center  pass  corticofugal  paths,  together  with  the  cen- 


Ant.  quadrigem. 


Fig.  142.— Diagram  of  the  optic  paths.     The  left  halves  of  the  retina  correspond  with  the 

right  halves  of  the  visual  field. 


tripetal  ones,  just  described,  into  the  same  ganglia,  from  which  the  optic 
radiation  arises. 

The  posterior  horn  is  surrounded,  on  the  outside,  by  three  medullary 
layers,  the  fibres  of  which  run  sagitally.     The  innermost,  lying  directly 


5io  DISEASES  OF  THE  BRAIN 

adjacent  to  the  ependyma  of  the  ventricles,  lining  the  ventricles  to  a  certain 
extent,  is  called  the  tapetum  (Strat.  sagittale  mediale). 

Towards  the  outside  of  it,  therefore  in  the  middle,  lies  the  stratum 
sagittale  internum  and  outside  of  it,  the  stratum  sagittale  externum  or  inferior 
longitudinal  fasciculus  (Fig.  143). 

The  latter  was  long  considered  a  pure  bundle  of  association  fibres  between 
occipital  and  temporal  lobes,  whereas  the  real  optic  radiation  (Gratiolet's) 
was  placed  in  the  stratum  sagittale  internum.  The  tapetum  was  supposed 
to  consist  of  corpus  callosum  fibres  exclusively.  But  it  has  been  proved, 
that  in  all  three  strata,  especially  in  the  stratum  internum  and  externum, 
optic  radiations  course,  that  the  stratum  sagittale  externum  also  contains 
association  fibres,  the  tapetum,  mostly  corpus  callosum  fibres,  by  inter- 
vention of  the  forceps.     At  any  rate,  the  optic  radiation  can  no  longer  be 


Tapetum.  \^         \_x/     \.  J) 

Strat.  sag.  med.  ^-A.      J  y^. S^~~^^  Fa.sc.  long,  inf. 

/  Strat.  sag.  ext. 

Gratiolet's  optic  radiation 
Strat.  sag,  int. 

Fig.  143. — Diagram  of  the  three  sagittal  medullary  layers  surrounding  the  posterior  horn  V 
in  a  frontal  cut  through  the  gyrangularis. 


confined  to  the  middle  layer,  but  to  the  inferior  longitudinal  fasciculus  must 
be  assigned  a  large  part  of  the  optic  projection  fibres. 

Lesions  behind  the  chiasm  throughout  the  visual  path,  that  is  in  the 
optic  tract,  in  the  ganglion  geniculatum  laterale,  in  the  optic  radiation  and 
the  cortical  center,  are  bound  to  cause  bilateral  homonymous-hemianopia, 
that  is  loss  of  function  in  the  halves  of  both  retinas,  on  the  same  side  as  the 
cerebral  lesion  and  correspondingly,  in  the  halves  of  the  field  of  vision  on  the 
opposite  side.  In  the  majority  of  cases,  the  whole  half  of  the  field  (except 
the  so-called  superfluous  field)  is  lost,  so  that  hemianopia,  in  the  strict  sense, 
occurs.  That  is,  when  the  focal  injury  is  on  the  left,  a  right  hemianopia  of 
both  eyes  corresponding  to  the  blindness  of  both  left  halves  of  the  retinas. 


THE  VISUAL  PATHS 


5ii 


Under  certain  conditions,  only  a  quarter  is  lost  for  both  eyes :  quarter  hemi- 
anopia  (Fig.  144). 

It  is  important  to  notice,  that  the  loss  of  exactly  half  of  the  field  of  vision 


Fig.  144. — The  location  of  the  three  sagittal  medullary  layers  in  a  horizontal  cut  of  the  brain 
(Drawn  from  a  Weigert  preparation  (3/2  of  natural  size)  from  Dejerine.)  C,  cuneus.  CA, 
Amnion's  horn.  Cc,  corpus  callosum.  Ce,  external  capsule.  Cex,  capsula  extrema.  Ci(y), 
capsula  interna  (genu).  Cip,  capsula  interna  (posterior  limb).  CI,  claustrum.  K,  calcarine 
fissure  (appears  in  two  places).  Lg,  gyr.  lingualis.  Put,  putamen  (external  layer  of  the  lentic- 
ular nucleus).  O,  occipital  convolution.  St.  s.  ext,  stratum  sagiltale  externum  or  fasciculus 
longitudinalis  inferior.  Str.  s.  i.,  stratum  sagittale  internum  or  Gratiolet's  visual  radiation. 
Dtr.  s.  m.,  Stratum  sagittale  mediale  or  tapetum.  T2,  second  temporal  convolution.  Th, 
thalamus. 


(total  hemianopia)  is  very  rare.  This  would  mean,  that  the  line  of  division 
between  the  parts  of  the  visual  field  that  are  retained  and  those  that  are  lost, 
would  go  as  a  vertical  line  directly  through  the  point  of  fixation.     It  occurs 


512 


DISEASES  OF  THE  BRAIN 


occasionally,  with  focal  injuries  in  the  optic  tract,  but  not  generally  with 
centrally  situated  focal  injuries.  Usually  at  least  a  small  area  about  the 
point  of  fixation,  in  the  direction  of  the  loss,  belongs  to  the  part  retained. 
This  very  area,  namely,  the  part  retained,  situated  beyond  the  vertical 


2S& 


Fig.  146. 
Figs.  145  and  146.— Visual  field  in  right-sided  hemianopia  with  residual  field  of  vision. 

meridian  passing  through  the  point  of  fixation,  is  known  as  the  "superfluous 
field  of  vision."  The  line  of  division  makes  a  curve  about  the  point  of 
fixation,  at  least,  which  with  its  convexity  extends  into  the  blind  half  of  the 
field  of  vision  (Figs.  145  and  146). 


THE  VISUAL  PATHS  513 

The  fact,  that  the  direct  vicinity  of  the  point  of  fixation  on  both  sides, 
is  almost  always  retained,  proves  that  the  region  of  clearest  sight  in  the 
retina  (macula)  is  double,  that  is,  represented  in  both  hemispheres.  The 
size  of  the  superfluous  field  of  vision  varies  with  the  individual  case  (2  to  15 
degrees)  and  may  even  be  different  in  each  eye.  Moderate  concentric 
narrowing  of  the  retained  field  of  vision,  also  may  occur,  more  obviously  in 
the  eye  that  is  on  the  opposite  side  from  the  focal  injury.  Certain  other 
irregularities  in  the  boundaries  of  the  visual  field,  in  focal  injuries  of  the 
occipital  lobe,  are  still  more  difficult  to  understand. 

Bilateral  hemianopia  is  called  cortical  blindness,  an  adopted,  but  not 
wholly  correct  nomenclature  (cerebral  blindness  is  better),  since  it  is  often  a 
question,  not  of  cortical  focal  injuries,  but  of  bilateral  interruption  of  the 
optic  radiation  or  cortical  focal  injury  on  one  side  and  subcortical  on  the 
other. 

In  this  bilateral  hemianopia  a  small  central  part  of  the  field  of  vision 
(sometimes  2  to  3  degrees)  is  generally  retained,  which,  however,  easily 
escapes  both  observer  and  patient,  and,  therefore,  demands  skilful  investi- 
gation to  prove  its  existence. 

In  rare  cases,  both  halves  of  the  visual  field  have  been  lost  simultaneously, 
by  embolism  of  the  occipital  lobes. 

Vision  in  the  retained  visual  field  is  often,  though  not  always,  diminished 
in  hemianopia  as  well  as  in  cortical  blindness. 

A  case  in  which,  on  the  contrary,  macular  vision  is  destroyed,  but 
peripheral  retained — that  is  a  field  of  vision  with  central  scotoma — due  to  an 
occipital  focal  injury— has  not  yet  been  observed  to  occur  as  a  permanent 
affection. 

The  fact  that  in  uniocular  as  well  as  in  binocular  hemianopia,  macular 
vision  is  usually  retained,  has  been  attributed  by  some  investigators  to 
double  cortical  representation  of  the  macula,  by  others  to  the  fact,  that  the 
situation  of  the  cortical  representation  of  the  macula,  is  protected  especially 
against  circulatory  disturbances,  by  others  (who  speak  against  an  isolated 
representation  of  the  macula),  to  a  representation  of  the  macula,  extending 
over  large  areas  of  both  occipital  lobes. 

From  the  last  point  of  view,  the  macula  has  an  extraordinarily  extended 
representation  in  the  occipital  lobe,  so  that  only  immensely  extended  focal 
injuries  could  rob  it  of  all  connections  with  the  cortex. 

How  far  definite  parts  of  the  retina  correspond  to  definite  parts  of  the 
cortical  visual  center,  i.  e.,  how  far  the  retina  is,  strictly  speaking,  projected 
on  the  cortex,  has  not  been  unanimously  decided.  There  is  much  difference 
of  opinion  about  it. 

As  the  termination  of  the  optic  radiation  (visual  sphere,  visual  center) 
chiefly  the  vicinity  of  the  calcarine  fissure  must  be  considered.  Especially 
its  upper  border — the  cuneus— and  its  under  border,  the  fusiform  gyrus, 

33 


5i4  DISEASES  OF  THE  BRAIN 

probably  the  entire  region  marked  out  by  the  Vicq  d'Azyr  stripe,  the  cellular 
structure  of  which  is  peculiar  and  uniform. 

The  fact  that  quarters  and  even  sixths  are  lost  by  focal  injuries  in  the 
optic  radiation  and  visual  center  proves,  at  any  rate,  that  no  complete  min- 
gling of  fibres,  which  come  from  various  parts  of  the  retina,  takes  place,  that 
at  least,  on  broad  lines,  projection  takes  place.  And  so  to  the  upper  quarters 
of  the  retina  in  both  eyes,  corresponds  the  upper  border  of  the  calcarine  fis- 
sure, to  the  lower  quarters,  the  lower  border. 

By  important  authorities,  the  pathological  discoveries  which  led  to  this 
proof,  were  used  to  show  that  not  the  cortical  focal  injuries  concerned,  by 
themselves,  but  accompanying  lesions  of  various  parts  of  the  optic  radiation, 
had  the  effect  mentioned,  so  that  a  projection  of  the  quarters  would  exist 
in  the  optic  radiation,  but  no  longer  in  the  cortex. 

The  last  word  has  not  yet  been  said  about  the  projection  of  the 
macula. 

Instead  of  hemianopia,  mere  disturbances  of  color  vision  may  affect  half 
the  field  of  vision  (hemiachromatopsia  or  hemidyschromatopsia).  Now 
general  weakness  for  color  vision  appears,  now  color  blindness  for  red  and 
green  only,  or  less  frequently  for  yellow  and  blue.  It  is  remarkable  that 
often  with  right  hemianopia,  is  found  hemidyschromatopsia  in  the  half  of 
the  field  of  vision  that  has  been  retained. 

Paralyses  of  the  muscles  of  the  eye  are  not,  per  se,  connected  with  hemi- 
anopia. But  the  loss  of  sensory-optic  stimulations  can  produce  an  uneco- 
nomic use  of  the  muscles  of  the  eye  and  hence  lead  to  mistakes  in  estimating 
distance. 

With  hemianopia,  from  whatever  cause  it  arises,  frequently  disturbance 
in  measuring  with  the  eye  is  connected,  in  such  a  way  that  by  halving  a 
horizontal,  the  half  corresponding  to  the  loss  in  the  field  of  vision  is  made 
too  small.     (The  opposite  error  is  rare.) 

Hemianopia  caused  by  a  focal  injury  in  the  optic  tract  must  be  distin- 
guished from  hemianopia  caused  by  an  interruption  in  the  optic  radiation  or 
injury  in  the  visual  center,  by  the  fact  that  in  the  former  case,  throwing  light 
upon  the  blind  half  of  the  retina,  causes  no  pupillary  reflex,  whereas  there  is 
no  cause  for  this  in  the  latter  case,  since  the  reflex  arc  (connection  of  the 
tract  with  the  oculomotor  nerve)  is  intact.  The  proving  of  pupillary  rigidity 
in  hemianopia  is,  however,  attended  with  great  difficulties,  because  it  is 
impossible  to  succeed  in  confining  the  illumination  exclusively  to  the  retinal 
halves  concerned.  But  in  bilateral  hemianopia,  caused  by  cortical  or 
subcortical  focal  injury,  the  fact  that  the  pupillary  reflex  is  retained,  can  be 
easily  proved. 

Subcortical  focal  injuries  may  irritate  the  visual  center,  and  occasionally 
cause  visual  hallucinations  in  those  parts  of  the  field  of  vision  that  have  been 
lost. 


AUDITORY  DISTURBANCES 


5i5 


It  is  still  uncertain  which  part  of  the  visual  system  is  affected  in  uraemic 
amaurosis. 

In  addition  to  the  hemianopsia  present  in  all  focal  diseases,  which  strike 
the  visual  system  from  the  tractus  to  the  visual  center,  it  is  found  frequently 
as  a  transient  affliction  in  progressive  paralysis,  after  the  attacks,  and  in 
migraine.  After  focal  injuries  in  the  temporal  and  parietal  lobes,  it  occurs 
temporarily  in  the  first  weeks  after  the  attack  as  an  indirect  symptom  of  the 
nearby  lesion.  Hemianopia  is  frequently  the  first  symptom  of  apoplexy. 
Focal  lesions  in  the  parietal  lobe,  especially  in  the  angular  gyrus,  which  ex- 
tend deeply  below  the  surface,  break  through  the  optic  radiation,  and  hence 
cause  permanent  hemianopia  (Fig.  147). 


$ts.L 


Fig.  147. — Case  of  softening  in  the  gyr.  angularis.  The  softening  focus  H  passes  through  the 
3  sagittal  medullary  layers.  Drawing  from  a  Pal  preparation.  Nat.  size  (frontal  cut).  At 
the  left,  medial  surface  of  the  brain.  V,  posterior  horn.  P.O.,  fissura  parieto-occipitalis.  The 
fissure  without  any  designation  below  the  latter  is  the  calcarine.  P,  forceps,  the  preserved  part 
of  the  corpus  callosum  radiation.  F.l.i.,  The  preserved  parts  of  the  fasciculus  longitudinalis 
inferior.    Str.s.i.,  Remains  of  the  stratum  sagittale  interius.     T,  place  of  the  absent  tapetum. 


2.  Auditory  Disturbances 

Auditory  Paths. — The  cochlear  nerve  (which  is  the  only  branch  of  the 
acoustic  nerve  that  is  concerned  with  hearing) ,  rises  in  the  cells  of  the  spiral 
ganglion  of  Corti.  It  ends  in  the  ventral  nucleus  of  the  cochlear  nerve  and 
in  the  dorsal  nucleus  (acoustic  tubercle-tuberculum  acusticum).  From 
these  spring  other  paths,  which,  excluding  further  interruptions  in  the  nuclei 
of  the  lateral  fillet,  for  the  most  part  pass  in  the  trapezoid  body  (interruption 
in  the  olive) ,  partly  pass  into  the  striae  acusticae,  on  the  other  side,  reach  there 
the  opposite  lateral  fillet,  and  for  the  most  part  end,  after  going  through  the 
arm  of  the  posterior  quadrigeminal  body,  in  the  medial  geniculate  ganglion 
(the  smaller  part  in  the  posterior  quadrigeminal  body)  (Fig.  148). 

Part  of  the  fibres  remain  uncrossed,  and  there  is,  besides,  a  commissure 
between  the  bilateral  nuclei  of  the  lateral  fillet,  so  that  each  cochlear  nerve 
is  connected  with  both  medial  geniculate  bodies,  hence  with  both  hemispheres. 
Here,  too,  we  find  se/mcrossing. 


5l6 


DISEASES  OF  THE  BRAIN 


From  the  medial  geniculate  ganglion  the  central  auditory  path  goes  to 
the  cortex  of  the  temporal  lobe  and  the  greater  number  of  the  auditory  fibres 
reach  the  transverse  convolutions  of  the  temporal  lobe  (gyr.  transversi), 
Heschl's  convolutions,  and  a  neighboring  tract  in  the  middle  third  of  the  first 
temporal  convolution. 

The  further  course  of  the  fibres  reaching  the  posterior  quadrigeminal 
body  is  a  matter  that  has  not  as  yet  been  cleared  up. 

Deafness  in  the  opposite  ear,  after  one-sided  lesions  in  the  temporal  lobe, 
has  never  been  observed.  In  that  case,  we  have  only  lessened  hearing,  in  both 
ears,  corresponding  to  the  connection  of  each  ear  with  both  temporal  lobes. 

Likewise,  unilateral  interruption  of  the  medial  geniculate  ganglionic 
paths,  going  to  the  temporal  lobe,  does  not  cause  total  one-sided  deafness. 


Gglgen.med         VorptereVierhuyel 

Hintere  Vierfiiigel 

Striae  acusticae 
dor,s  AT  cochL 


Later  ale  /Schteift 


Cortisckes  Organ 
McocAlearis 


tfYL  Corpus  trapezoides 

Fig.  148. — Scheme  of  the  central  auditory  paths.  Interruption  in  the  nuclei  of  the  lateral 
fillet  not  considered.  N.N.  VI.,  nucleus  of  the  abducens.  O.d.,  right  superior  olive.  In  the  cut: 
Laterale  Schleife  =  lateral  fillet.  Scblafenlappen  =■  temporal  lobe.  Querwindung  =  transverse 
convolution.  Vordere  Vierhigel  =  anterior  quadrigeminal  bodies.  Hintere  Vierhugel  =  posterior 
quadrigeminal  bodies.     Cortisches  Organ  =  Corti's  organ. 

Total  deafness  in  both  ears  may  be  caused  by  bilateral  disease  in  the 
temporal  lobes  (so-called  cortical  deafness).  Heretofore,  this  has  been 
observed  only  in  very  extended  focal  injuries,  so  that  the  above  anatomical 
boundaries  of  the  auditory  center  (based  upon  the  myelogenetic  method) 
could  not  be  clinico-pathologically  substantiated,  and  many  authors  ascribe 
to  the  auditory  center  much  more  space  in  the  temporal  lobe,  than  in  follow- 
ing Flechsig,  is  given  above. 

More  often  than  total  deafness,  great  difficulty  in  hearing,  in  both  ears, 
results  from  bilateral  lesions  of  the  temporal  lobes.  This  does  not  show  the 
well  known  tone-gaps  of  labyrinthine  diseases  (as  the  loss  of  the  highest 
tones),  but  a  more  regular  diminution  for  the  entire  tonal  sequences. 

This  does  not  decide  against  a  projection  of  various  tone-heights  on  the 
cortex,  since  in  the  two-sidedness  of  the  representation  of  the  organs  of  Corti 


AUDITORY  DISTURBANCES 


5*7 


in  both  temporal  lobes,  an  insulated  tone-gap  could  only  appear,  if  by  chance, 
exactly  the  same  parts  were  destroyed  on  both  sides.  At  any  rate  the  assump- 
tion, that  is  to  be  discussed  under  sensory  aphasia — that  the  cortical  end- 


Insul 


^Auditory 
'tract  to 
cortex. 


First 

temporo 
sphen- 
oidal lobe 


Optic  plus 


Organ  of  < 
Corti 

Nucl.  vent 


X.  cochl. 
Fibres  of  the 
trapezoid  body 


long.  int. 


Stria?  acustics 


Fig.  149.— Topography  of  the  auditor}'  paths.     (A  connection  is  shown  between  the  unmasked 
posterior  quadrigeminal  body  and  the  C.  gen.  int.)    In  the  cut:  Linsenk.  =  lenticular  nucleus,    tot. 

Schl.  =  lower  fillet. 

ing  of  the  fibres  of  the  tones  b'  to  g"— a  very  important  part  of  the  tonal 
sequence— take  up  a  definite  spacial  division  within  the  entire  auditory 
sphere,  is  as  yet,  wholly  hypothetical. 

Auditory  disturbances  caused  by  focal  injuries  in  the  cap  of  the  cerebral 


5i8  DISEASES  OF  THE  BRAIN 

peduncle  and  the  pons  and  in  the  region  of  the  posterior  quadrigeminal 
body,  occur,  yet,  because  of  the  wide  distribution  of  the  paths  concerned, 
and  their  semi-crossing,  are  seen  permanently  in  high  degree,  only  when  the 
focal  injury  is  very  extensive — except  when  nucleus  or  root  of  the  cochlear 
nerve  is  destroyed,  in  which  case,  deafness  in  one  ear  results. 

3.  Olfactory  and  Gustatory  Disturbances 

In  basal  focal  injuries  that  affect  the  olfactory  nerve,  nucleus,  or  tract, 
the  olfactory  trigone,  or  the  perforated  substance,  a  diminution  in  the 
sense  of  smell,  on  both  sides,  is  noticed. 

The  olfactory  centers  lie  in  the  hippocampal  gyrus  and  the  Ammon's 
horn. 

Olfactory  hallucinations  also  appear  in  irritations  of  the  olfactory  regions. 

The  gustatory  sphere,  too,  lies  in  Ammon's  horn,  the  hippocampal 
gyrus  and  the  adjacent  posterior  end  of  the  fornicatus  gyrus.  The  peripheral 
paths  of  taste  course  in  the  trigeminus  and  the  glosso-pharyngeal  nerves. 

3.  Mnemic-associative  Derangements 

(a)  Aphasic  Disturbances 

1.  Introduction 

Disorders  of  the  Projection  System  of  the  Apparatus  of  Speech: 
Anarthria  in  Bulbar  and  Pseudo-bulbar  Paralysis 

Language  is  a  system  of  symbols  serving  for  the  communication  of 
thought  between  men. 

The  symbols  of  spoken  language  are  sounds  and  combinations  of  sounds 
which  are  naturally  produced  by  the  action  of  the  muscles  of  the  lips,  jaws, 
tongue,  palate,  and  larynx.  In  this  aspect,  speech  is,  then,  a  series  of  move- 
ments, the  effect  of  which  is  to  give  other  people  symbols  of  the  psychic 
processes,  such  as  thoughts,  feelings,  desires,  etc.,  of  the  person  speaking. 
{Speech-expression.) 

But  considered  with  reference  to  its  effect  as  audible  sound,  it  may  be 
included  with  the  process  of  hearing,  and  evokes  in  the  listener  the  corre- 
sponding psychic  processes.  The  role  of  the  listener  is  understanding. 
{Speech-perception.) 

Not  until  late  in  the  development  of  both  the  species  and  the  individual, 
do  we  find  a  second  system  of  symbols:  written  speech  which  also  has  an 
expressive  (writing)  and  a  receptive  (reading)  side. 

Both  written  and  spoken  language  employ  certain  muscles  which  serve 
other  purposes  as  well.  Thus  the  muscles  of  the  tongue,  jaws,  and  lips, 
serve  for  eating,  chewing,  sucking,  swallowing,  licking,  kissing,  etc.,  and 
for  various  voluntary  and  involuntary  imitative  movements. 


SPEECH  DISTURBANCES 


5i9 


If  this  system  of  muscles  or  its  corresponding  nervous  centers  is  diseased 
(paralysis,  paresis,  ataxia,  tremor,  etc.) ,  speech  is  affected  secondarily.  The 
resulting  disturbances  of  speech  are  called,  according  to  their  degree,  dys- 
or  an-arthria. 

To  this  nervous  mechanism  belong: 

1.  The  cortical  centers  of  the  muscles  concerned,  therefore  the  centers 
of  the  12th,  7th,  10th,  and  nth  Nerves  in  the  lower  fourth  of  the  anterior 
central  convolution.     (Operculum  Rolandi.) 

2.  The  motor  (cortico-bulbar)  tract  from  these  centers  to  the  bulbar 
nuclei  of  the  nerves.  They  pass  through  the  white  matter,  through  the 
"genu"  of  the  internal  capsule,  the  base  of 
the  cerebral  peduncle  and  reach  to  the  nu- 
clei of  both  sides  in  the  pons  Varolii  and  the 
medulla  oblongata. 

Because  of  the  connection  of  each  hemi- 
sphere of  the  brain  with  the  nuclei  of  both 
sides,  it  results  that  supranuclear  lesions  of 
one  side  do  not  produce  permanent  paraly- 
sis of  the  nerves  concerned,  and  therefore 
the  dysarthria  resulting  is  neither  severe 
nor  long-continued.  A  break  in  the  con- 
nection of  the  cortico-bulbar  tracts  and 
their  centers  can  indeed  bring  about  tem- 
porary dysarthria,  especially  when  the  left 
hemisphere  is  affected.  Slight  dysarthric 
disturbances  often  persist  after  a  lesion  of  one  side. 

3.  The  peripheral  tract  from  the  nucleus  of  the  12th  Nerve,  etc.,  to  the 
muscle. 

Disease  in  the  medulla  oblongata  affects  the  peripheral  tract  represented 
by  3.  If  the  bulbar  nuclei,  or  their  roots,  are  destroyed,  we  have  bulbar 
paralysis,  together  with  atrophy  and  degenerative  reactions  in  the  muscles 
concerned  (tongue,  lips).  It  causes  besides  the  dysarthria — which  consists 
of  incorrect  articulation  of  letters,  and  is  conditioned  by  the  centers  affected, 
whether  they  be  more  particularly  labial,  palatal,  or  lingual — other  paralytic 
phenomena:  partial  paralysis  of  the  facial  muscles  and  disturbances  in  eating, 
chewing  and  swallowing. 

If  the  cortical  centers  or  the  supranuclear  nerve  tract  are  affected  on  one 
side  alone,  there  results  only,  as  we  have  said,  a  slight  degree  of  disturbances 
in  the  muscles  concerned:  slight  paralysis  of  the  ends  of  the  facial  nerve, 
deviation  of  the  tongue,  when  it  is  extended,  slight  difficulty  in  articulation 
with  no  disturbance  in  swallowing,  no  atrophy  and  no  reaction  of  degenera- 
tion. But  if  the  centers  or  nerve  tracts  are  disturbed  on  both  sides,  or  on  one 
side  the  center  and  on  the  other  the  tract,  similar  disturbances  result  as  arise 


Km         ' — ■*-  xjz 

Fig.  150. — Seats  of  lesions  causing 
pseudo-bulbar  paralysis,  i  and  2  foci 
in  the  left  and  right  cortical  center  of  the 
seventh,  twelfth  nerves,  etc.  3  and  4  foci 
in  the  paths  to  the  pontine  and  bulbar 
nuclei.  The  combinations:  1  +  2,  2  + 
3,  1  +  4,  3  +  4  produce  pseudo-bulbar 
paralysis. 


52o  DISEASES  OF  THE  BRAIN 

from  the  destruction  of  the  bulbar  nuclei  themselves,  only  that  atrophy  and 
degenerative  reaction  are  absent  and  that  there  is  a  smaller  degree  of  dis- 
turbance. Since  the  supranuclear  fibres  above  the  pons  disperse  in  all 
directions,  very  great  focal  injury  is  necessary  to  interrupt  them  wholly. 
Those  localized  in  a  supranuclear  position  in  the  pons  are  most  likely  to  effect 
this  (Fig.  150). 

Since  such  two-sided  disturbances  of  the  cortical  centers  and  the  cortico- 
bulbar  nerve  tracts  produce  phenomena  so  like  bulbar  paralysis,  they  have 
been  named  pseudo-bulbar  paralyses. 

The  essential  feature  of  pseudo-bulbar  paralysis  is  therefore  that  on 
both  sides  the  cortico-bulbar  nerve  tracts  (eventually  at  the  place  of  their 
cortical  origin)  are  interrupted  at  some  one  place.  To  bring  this  about, 
unless  it  be  in  the  pons  the  nerve  tracts  must  be  stricken  twice,  once  on  the 
left,  and  once  on  the  right  side.  In  this  case,  there  are  disturbances  in  articu- 
lation, in  swallowing,  etc. 

Disturbances  in  this  efferent  neuro-muscular  apparatus  belong  to  the 
derangement  of  the  projection  system  alone  and  have  no  connection  with 
aphasia,  even  when  they  are  caused  by  cortical  injuries.  They  cause  corti- 
cal, cortico-bulbar  and  bulbar  anarthria  or  dysarthria. 

Not  only  spoken  but  written  language  as  well  employs  a  neuro-muscular 
apparatus,  which  serves  other  purposes  as  well.  It  is  the  apparatus  which 
innervates  the  more  delicate  movements  of  the  hand.  Paralysis,  weakness, 
ataxia  of  the  hand,  produce  the  same  hindrances  in  writing,  that  pseudo- 
bulbar or  bulbar  paralysis  produces  in  uttered  speech,  except  that  only 
rarely  is  the  other  hand  affected  also,  and  that  the  movements  used  in  writing 
can  be  performed,  more  or  less,  by  any  other  movable  portion  of  the  body 
(foot  or  tongue),  while  the  production  of  sound  is  confined  to  a  definite 
muscular  system.  Just  as  utterance  can  be  affected  indirectly  by  injury  to 
the  efferent  neu.ro-muscular  apparatus,  the  perceptive  faculties  of  speech 
suffer  from  lesions  of  the  perceptive  apparatus,  i.  e.,  of  the  organs  of  hearing 
and  seeing.  By  a  difficulty  in  hearing  verging  on  deafness,  or  as  we  shall  see, 
by  a  definite  gap  in  the  perception  ofto-nes,  the  understanding  of  speech  can  be 
destroyed,  as  well  as  the  ability  to  read  by  a  weakness  of  eyes,  verging  on 
blindness. 

Even  then,  we  cannot  speak  of  aphasic,  but  of  perceptive  disturbances. 

2.  Disturbances  of  the  Mnemic-as  so  dative  Speech  Apparatus:  Aphasia. 

Internal  Speech 

When,  however,  the  cortico-bulbar  apparatus,  on  which  sound-formation 
depends,  fulfills  all  its  functions,  and  yet  the  patient  can  only  speak  badly  or 
not  at  all,  aphasia  exists.1 

1  Mutism  from  hysteria  and  mental  disorders  is  not  taken  into  consideration  here. 


MECHANISM  OF  SPEECH  521 

What  has  failed,  is  so-called  internal  speech,  i.  e.,  something  that  has  been 
learned,  a -possession  of  memory.1 

We  use  internal  speech  to  distinguish  it  from  external  speech.  The 
latter  is  the  speech-musculature's  creation,  which  produced  by  the  one,  the 
other  hears.  The  primary  condition  for  external  speech  is  internal  speech, 
but  loss  of  internal  speech  entails  the  loss  of  external  speech. 

Internal  speech  is  ever  present  while  we  are  thinking.  Most  of  our 
thoughts  present  themselves  in  the  form  of  speech.  In  the  case  of  vivid 
thought  impulses  discharge  themselves  into  the  neuro-muscular  apparatus, 
so  that  we  have  abortive  innervations,  which  may  rise  into  involuntary 
utterance  of  thought. 

The  internal  word  consists  originally  of  two  parts,  closely  connected: 

1.  Memory  of  the  word-sound,  or  the  acoustic  word  (a),  the  residuum  of 
remembered  words,  that  the  child  hears  from  his  environment.  It  is  the 
oldest  word-possession  and  the  great  majority  of  words  are  acoustically 
acquired. 

Moreover  the  meaning  of  the  word,  i.  e.,  the  corresponding  concept, 
attaches  itself  to  the  acoustic  word — as  the  word  Bow-wow  to  the  concept 
of  the  dog — so  that  for  many  words,  before  the  child  itself  can  speak, 
there  arises  a  close  connection  between  the  concept  (c)  and  the  acoustic 
word.     C — a  is  the  first  speech-association. 

2.  The  memory  of  word-movements  or  the  motor-word  (m).  The 
memory  of  the  process  necessary  for  the  utterance  of  the  word. 

Usually  this  process  is  called  kinesthetic.  It  comprehends  memory  of 
perceptions  reaching  all  the  parts  moved  by  us  in  speech,  lips,  tongue, 
palate,  etc.  The  utterance  of  the  word  '"parrot"  gives  us  other  sensations 
of  place,  movement,  and  contact  than,  say,  the  word  "weasel." 

In  truth,  we  find  in  consciousness  nothing  besides  memories  of  this 
perception  complex.  But  beneath  the  threshold  of  consciousness  there 
comes  a  richer,  a  more  material  memory  possession.  The  frequently 
repeated  sequence  of  innervations,  which  correspond  with  a  definite  sound- 
complex,  leaves  probably  a  lasting  connection  between  the  co-working 
innervational  elements,  so  that  the  motor-word2  is  composed  of  kinassthetic 
and  innervational  memories,  of  which  only  the  former  have  a  psychic 
equivalent. 

The  motor-word  is  greatly  dependent  on  the  acoustic  word.  The  mouth 
cannot  produce  what  the  ear  has  not  heard.  The  child  learns  to  repeat  a 
series  of  words  he  has  heard.  By  this  means  a  very  close  connection  is 
produced  between  a  and  m  (a — m).    This  is  the  word  as  it  exists  in  the  child. 

1  We  have  learned  articulation,  too.     But  it  is  not  what  we  have  learned  that  is  lost  in  dysar- 
thria, but  the  natural  instrument,  with  which  what  has  been  learned  is  produced. 

2  This  innervational  memory  holds  good  in  part  for  whole  words,  especially  however,  for  parts 
of  words,  for  syllables  and  letters. 


522  DISEASES  OF  THE  BRAIN 

This  combination  of  the  acoustic  with  the  motor-word  represents  internal 
speech  in  its  restricted  sense.  As  we  see,  the  concepts  are  predominantly 
connected  with  a,  and  with  m  only  through  the  mediation  of  a,  so  that  the 
relations  of  the  motor-word  to  the  concept  is  brought  about  mainly  through  a. 
The  following  association  is  thus  formed: 

C 

TI 

(a — m)    . 

The  recollection  of  word-movements  can  combine  itself  with  the  concept 
only  secondarily,  inasmuch  as  every  concept  has,  indeed,  led  to  m  only 
through  the  mediation  of  a,  yet  the  frequent  simultaneous  appearance  of  C 
and  m  nevertheless  produces  a  secondary  direct  association.  This  less 
firm  and  comprehensive  connection  we  shall  represent  by  a  dotted  line 
between  C  and  m. 

C 

x\ 

(a— >m) 

When,  between  the  ages  of  seven  and  eight  years,  the  child  learns  the 
written  language,  by  the  complex  (a — m)  the  written  word  attaches  itself  to 
the  sounded  word.  But,  unlike  hieroglyphics,  the  written  word-symbols 
are  not  direct  symbols  for  the  apprehension  of  the  objects,  but  are  symbols 
for  sounds,  i  .e.,  for  parts  of  the  sounded  word.  In  other  terms,  our  writing  is 
phonetic. 

Now  only  does  the  child  learn  to  separate  the  words  into  syllables  and 
letters  and  to  assign  to  each  letter  a  written  symbol.  Hence  it  is,  that  as  a 
rule,  the  written  language  is  connected  with  the  concept  only  through  the  mediation 
of  the  sounded  language.  To  each  sound  an  optical  letter  symbol  is  assigned 
(o)  and  in  writing  this  optical  letter  is  produced  by  the  movement  of  what- 
ever member  is  guiding  the  pen.  Thus  the  acoustic  motor-word,  aroused 
by  the  concept,  is  separated  into  its  component  parts,  each  of  which  evokes 
its  corresponding  optical  image,  from  which  impulses  flow  to  the  hand  center. 

Through  practice,  graphic-motor  recollections  are  formed  in  the  hand- 
center. 

The  complex  associations  formed  thus  between  the  concept  and  the 
acoustic,  linguo-motor,  optical  and  grapho-motor  components,  represents 
internal  speech  in  its  wider  sense. 

Now  impulses  flow  from  m  to  the  neuro-muscular  apparatus  of  the 
tongue  muscles.  The  stimulations  in  the  hand-center  flow  off  to  the 
muscles  of  the  hand,  while  to  a  and  o  arrive  the  centripetal  stimulations  of 
the  ear  and  eye.  The  sound  of  the  words  penetrates  to  the  seat  of  the 
acoustic  memory  images,  arouses  them,  and  thereby  brings  about  the 
release  of  the  components  associatively  connected  with  a,  that  is  of  the 
whole  word. 


MECHANISM  OF  SPEECH 


523 


The  theory  once  held  of  a  direct  speech  pathway  to  and  from  the  bulbar 
nuclei  had  to  be  abandoned.  On  the  contrary  speech  makes  use  of  the 
same  projection  pathways  (although  with  a  certain  preference  for  those  on 
the  left  side)  as  the  other  movements  of  the  tongue,  lips,  etc.  Therefore 
the  stimulation  from  m  must  first  reach  the  cortical  center  "  p"  of  the  7th 
and  1 2th  Nerves,  etc.,  in  the  Rolandic  Operculum,  where  the  real  innervation 
takes  place.  It  is  consequently  necessary  to  insert  " p"  between  m  and 
tongue. 

In  the  same  way  the  acoustic  stimulations  reach  first  the  acoustic  pro- 
jection-field "alpha"  before  waking  the  remembrances  in  a.  Even  the 
authors  who  do  not  share  the  view  of  Flechsig  of  the  absolute  separation  of 
the  projection-field  (acoustic-center)  from  the  mnemic  field  (a),  assign  the 
perceptive  and  the  mnemic  process  to  various  processes  of  the  same  field, 
so  that  perception  and  waking  of  the  memory  are  regarded  as  two  distinct 


Fig.   151. — B,  conception;  a,  acous- 
tic component;  m,  speech  motor  com 
ponent;  0,  optic  component;  gr,  grapho- 
motor component. 


Ear        Eye  Hand  Tongue 

Fig.  152 — a,  acoustic  perception;  u,  in- 
nervation. The  other  letters  as  in  scheme 
Fig.  151. 


parts  of  the  process.     (For  o  and  gr  the  corresponding  projection  apparatus 
are  not  shown  separately.) 

The  complete  scheme  of  speech  is  shown  in  Fig.  152. 

This  scheme  which  is  not  intended  to  represent  anatomic  relations,  but 
to  make  evident  and  comprehensible  certain  fundamental  rules,  plainly 
teaches  the  following:  the  way  by  speaking  and  writing  leads  from  C1 
principally  over  a. 

Speaking: — :B — a — m — "  jj,  " — tongue 

Writing: — :B —  /  a  \  \ 

(    I    )  yo — gr — hand 

In  the  same  way  not  only  in  understanding  speech  (Ear — "alpha" — a — B), 
but  also  in  reading  (Eye — o — a — B)  the  way  leads  over  a. 

V 

m 

Hence  it  is  clear  that  an  annihilation  of  a  disturbs  not  only  speech  but 
'B  =  Concept  is  represented  in  the  above  scheme  only  by  a  point,  but  in  the  brain,  very 
extensive  cortical  areas  correspond  to  it. 


524  DISEASES  OF  THE  BRAIN 

also  reading  and  writing.  But,  since  in  writing  and  reading  a  dismembering 
of  the  entire  word-sound  (a — m)  into  its  component  parts  (syllables  and 
letters)  takes  place,  also  the  loss  of  m  will  be  peculiarly  damaging  for  reading 
and  writing.  Writing  and  reading  depend  upon  the  condition  that  a  and  m 
are  intact! 

These  relations  of  dependence  (especially  that  B  be  directly  connected 
only  with  a,  further,  that  o  enter  into  connection  with  B  only  over  a — m) 
are  not  fixed  inevitably  by  the  organization  of  the  brain,  but  by  the  way  the 
function  of  speech  is  learned. 

The  deaf-mutes  are  the  best  illustration  of  this  as  they  learn  to  write  and 
even  to  speak,  though  a  does  not  exist  in  them  at  all. 

The  relations  of  dependence  can  therefore  be  modified  by  individual 
predisposition,  or  peculiar  development.  For  a  time,  it  was  believed  even 
that  individual  peculiarities,  sometimes  the  visual,  then  the  acoustic,  and 
again  the  motor  predisposition  gave  grounds  for  questioning  the  regularity 
of  speech  mechanism  altogether,  that  a  great  number  of  men  with  motor 
predispositions  (Moteurs)  could  suffer  the  loss  of  acoustic  speech  com- 
ponents without  injury  to  their  speaking,  so  that  they  found  innervations  to 
speech  directly  from  conception  (i.  e.,  in  other  words:  the  Concept  evokes 
the  motor  components).  Or  that  a  great  number  of  optically  predisposed 
men  (Visuels)  are  not  disturbed  in  reading  when  a  is  injured,  since  they 
would  be  in  a  condition  to  evoke  directly  from  conception  visual  images  of 
letters.  But  it  has  been  proved  that  the  great  fundamental  relations,  in  con- 
sequence of  similar  formation  in  the  majority  of  men,  correspond  more 
closely,  than  the  exaggerated  observations  of  individual  differences  have 
caused  some  to  think.  One  can  not  even  count  four  approximately  similar 
types.  There  are  only  a  few  exceptions  to  the  fundamental  relations  given 
in  the  scheme.  The  predominating  place  of  a  is  almost  always  preserved  and 
not  overbalanced  by  individual  predisposition,  so  that  the  motor  word  in  the 
majority  of  cases,  is  conditioned  by  the  connection  between  a  and  B,  and 
written  language  usually  also  depends  upon  a.  On  the  other  hand,  the  rule 
that  written  language  depends  also  on  m,  has  not  a  few  exceptions.  There 
are  people  who  suffer  the  loss  of  motor  memories,  without  their  written 
language  being  affected. 

For  the  quicker  or  slower  restoration,  for  the  finer  differentiation,  and 
for  the  degree  of  the  disturbances,  individual  differences  must  be  considered. 
So  must  the  fluency  in  writing,  which  a  man  possessed  before  illness,  be  con- 
sidered also.  As  a  rule,  however,  we  may  assume  an  approximate  similarity 
in  the  organization  of  different  human  brains. 

3.  The  Speech  Regions  in  the  Brain 

Let  us  see,  now,  how  the  psychological  scheme  is  carried  out  in  the  brain. 
Here,  before  all,  a  fundamental  fact  is  to  be  noted:  that  the  speech-functions 


CEREBRAL  SPEECH  CENTERS 


525 


are  performed  chiefly  by  the  left  hemisphere.  The  opposite  holds  good 
only  in  left-handed  people,  in  whom  the  right  hemisphere  plays  the  greater 
part. 

Besides  the  left-handed  people,  4  to  6%  are  ambidextrous — using  both 
hands  equally  well — and  only  in  these  do  both  hemispheres  share  equally 
in  the  functions  of  speech.  Now,  it  must  not  be  thought  that  in  right- 
handed  people — the  majority  of  mankind — the  right  hemisphere  not  only 
takes  no  part  whatsoever  in  speech,  but  is  also  by  nature  unfitted  for  the 
function  of  speech.     This  is  not  true. 

The  right  hemisphere,  may,  in  healthy  people,  share  in  speech  to  a 
certain  extent;  but  it  is  not  able,  by  itself,  to  care  for  the  functions  of  speech, 
so  that  from  disturbance  in  certain  parts  of  the  left  hemisphere,  speech  in 


Ft 


Mot. A.  ro- 


p  /  Op.R 

J.  ope  re. 


Cfyr  temp,  prof 


Al.+Agn 

OeilS.Ji 

Fig.  153. — -The  speech-region  (red).  Mot.A ,  motor  aphasia.  Insel.A,  insular  aphasia.  Sens. 
A,  sensory  aphasia.  A  focus  in  X  (in  the  temporal  transverse  convolution)  may  perhaps  cause 
pure  word  deafness.    Al.  Agr.,  Alexia  and  agraphia. 


certain  of  its  functions  (expressive,  perceptive),  is  destroyed  or  badly  injured. 
Were  the  right  hemisphere  as  important  to  speech  as  the  left,  the  same 
results  would  follow  a  lesion  in  the  right  hemisphere,  which  is  not  the  case. 
Besides  participating  to  a  certain  degree  in  the  functions  of  speech  in  the 
healthy  individual,  the  right  hemisphere  may,  after  a  lesion  in  the  left,  under- 
take to  some  extent,  after  practice,  the  function  of  the  injured  half.  There 
is,  however,  never  a  complete  assumption  in  the  case  of  right-handed  people, 
and  defects  can  always  be  proved  by  delicate  tests. 

All  the  rules  to  be  stated  hereafter  hold  good  with  only  these  limitations: 

1.  That  ambidextrous  people  are  not  included. 


526  DISEASES  OF  THE  BRAIN 

2.  That  the  right  hemisphere  (the  left  in  the  left  handed)  in  the  course 
of  time,  can  to  a  certain  extent,  to  be  discussed  later,  substitute  for  the  left. 

3.  That  no  individual  differences  in  predisposition  or  use  exist. 

In  the  left  hemisphere  there  is  an  area  that  is  principally  concerned  with 
the  functions  of  speech:  the  speech  region,  to  which  are  to  be  added,  those 
regions  in  which  lesions  under  the  cortex  result  in  serious  losses  of  function. 
But  since  the  most  varied  combination  of  breaks  in  the  fibres,  so  numerous 
as  not  to  be  discussed  in  a  short  space,  cause  disturbances  in  speech,  a 
diagram  of  the  speech  region  on  the  surface  can  give  only  a  rough,  approximate 
representation  (Fig.  153).  Only  the  names  of  the  most  important 
speech  disorders  can  be  marked  upon  it.  If  all  the  regions  in  which  lesions 
produce  difficulty  in  finding  words  (amnesic  aphasia)  were  included,  the 
region  for  speech  would  have  to  be  drawn  considerably  larger. 

The  speech  region,  therefore,  comprehends  the  lower,  posterior  part  of  the 
convexity  of  the  left  frontal  lobe,  the  island  of  Reil,  the  front  portion  of  the 
operculum  of  the  anterior  cerebral  convolution  {the  Rolandic  operculum) ,  the 
posterior  third  of  the  temporal  lobe,  and  one  part  of  the  lower  occipital  lobe. 

These  various  parts  of  the  speech  region  are  not  of  equal  importance. 

4.  Complete  Motor  Aphasia 
{Cortical  Motor  Aphasia  of  Wernicke) 

We  must  distinguish  the  Frontal  speech  region,  the  center  nucleus1  of 
which  is  the  posterior  two-thirds  of  the  inferior  frontal  convolution  (Pars 
triangularis  and  opercularis,  the  latter  being  Broca's  region),  but  which,  at 
least  in  many  people  extends  to  the  lower  third  of  the  anterior  central  gyrus 
and  the  front  part  of  the  Island  of  Reil,  probably  even  up  to  the  middle 
frontal  convolution.  And,  moreover,  the  anterior  and  posterior  boundaries 
seem  to  differ  somewhat  in  different  individuals.  Extensive  lesions  of  this 
region  cause  disorder,  which,  clinico-psychologically,  corresponds  to  the 
loss  of  motor  word-components.  The  patients  are  still  able  to  understand 
what  is  being  said  to  them — a  is  retained— but  can  not  find  the  innervation- 
complexes  necessary  to  the  utterance  of  words — that  is,  motor  mnemic 
images  are  lacking.  They  are  therefore  unable  to  utter  words  {word-dumb, 
aphemic),  or  have  left  only  a  few  fragments  of  speech.  Because  of  the 
dependence  of  written  speech  on  m,  writing  is  lost  or  badly  disturbed  in 
the  majority  of  people  (see  below) — except  copying,  which  over  o gr 

eye  hand 

is  possible  without  the  use  of  (a — m).  Even  reading  shows  a  gradual  but 
very  distinct  deterioration.  Reading  aloud  is  impossible  because  of  the 
inability  to  utter  words,  but  the  internal  comprehension  of  reading  is,  for  the 
most  part,  not  seriously  affected,  although  seldom,  intact. 

1  Naturally,  the  word  "nucleus"  here,  has  nothing  in  common  with  the  anatomical  concept 
of  "nucleus." 


MOTOR  AND  SEXSORY  APHASIA  527 

The  resulting  clinical  picture  is  known  as  complete  motor  aphasia,  con- 
sisting of: 

Cessation  of  imitative  speech. 

Cessation  of  spontaneous  speech. 

Cessation  of  reading  aloud. 

Cessation  of  spontaneous  writing. 

Cessation  of  writing  from  dictation. 

Difficulty  in  the  comprehension  of  reading. 

Comprehension  of  speech  and  copying  are  retained. 

In  mental  excitement,  the  sufferer  from  motor  aphasia  (in  contrast  to  the 
anarthric)  at  times  correctly  utters  a  word  or  even  a  sentence  which  as  a  rule 
he  cannot  bring  to  utterance.  The  speech  of  many  people  thus  affected 
shows  improvement  when  singing.  (Speech  as  an  expression  of  the  emotions 
may  be  partly  retained,  when  as  an  expression  of  thought  it  is  lost.  Thus 
the  motor-aphasic  may  swear  roundly.) 

For  the  retrogression  of  motor  aphasia,  and  the  peculiarities  of  returning 
speech,  cf .  page  529. 

For  clinical  pictures  approximating  complete  motor  aphasia,  cf.  under 
insular  aphasia,  page  530. 

5.  Complete  Sensory  Aphasia 

{Cortical  Sensory  Aphasia  of  Wernicke) 

On  the  other  hand,  extensive  focal  injury  of  the  temporal  speech-region 
(the  posterior  third  of  the  superior  temporal  convolution  with  the  temporal 
transverse  convolution  and  the  adjoining  anterior  part  of  the  supramarginal 
gyrus)  produces  sensory  aphasia  (cf.  also  pages  531,  534,  536). 

In  sensory  aphasia,  the  memory  of  word-sounds  (a)  is  severely  impaired. 
Consequently,  the  main  symptom  is  word-deafness.  The  patients  hear,  but 
fail  to  understand  what  is  said  to  them.  And  since  the  sound  of  the  word  is 
not  grasped,  of  course  the  meaning  of  the  word  is  not  understood.  To  grasp 
the  sound  of  the  word,  more  is  needed  than  to  hear  each  separate  tone. 
Besides  the  formation  of  letters  and  syllables  from  the  tones,  and  the  ability 
to  grasp  them,  the  co-operation  of  the  memory  of  word-sounds  is  particularly 
necessary. 

Since,  as  we  saw,  acoustic  components  play  a  great  part  in  speech,  it  is 
disordered  by  injury  to  them. 

There  is  not  indeed  a  cessation  of  speech,  as  in  motor  aphasia,  but  there 
are  many  derangements,  and  much  confusion  of  words,  syllables,  and  letters, 
known  as  Paraphasia.1 

If  the  patient  instead  of  knife  says  hammer,  he  has  verbal  paraphasia;  if, 

1  In  the  derangements  of  the  sensory-aphasic  patient,  "getting  stuck"  plays  an  important 
part. 


528  DISEASES  OF  THE  BRAIN 

instead  of  knife,  he  says  life,  he  has  literal  paraphasia.  If  the  latter  is  highly 
developed,  the  result  is  an  incomprehensible  jargon:  a  jargon-par  aphasia, 
where  one  can  understand,  only  by  conjecture,  what  the  patient  means. 

The  degree  of  paraphasia  varies  greatly. 

Many  patients,  suffering  from  sensory  aphasia,  can,  by  circumlocutions, 
avoid  the  words  they  do  not  command,  and  utter  a  lot  of  phrases,  which 
while  correct  in  themselves,  are  conspicuous  by  their  lack  of  concreteness. 
Then  the  serious  disturbances  in  speech  appear  only  when  one  lets  them  name 
concrete  objects  (knife,  chair,  window,  etc.). 

The  sensory  aphasic  in  contradistinction  to  the  motor  aphasic,  even  in 
an  advanced  degree  of  speech-disturbance,  commands  a  great  number  of 
letters  and.  syllables,  the  expression  of  which  causes  him  no  trouble;  but  he 
does  not  always  use  the  fitting  ones,  and  often  combines  them  so  confusedly, 
as  to  be  understood  only  partially,  or  not  at  all. 

Such  a  patient  is  even  usually  talkative  (scarcely  realizing  that  his  speech 
is  unintelligible),  while  the  sufferer  from  motor  aphasia  generally  remains 
silent,  hesitating  to  attempt  speech  (noticing  his  errors  because  of  the  intact 
acoustic  word  (a)). 

Since  written  speech  depends  on  a,  it  is  disturbed  even  in  sensory  aphasia, 
the  writing  being,  for  the  most  part,  better  than  in  the  case  of  motor  aphasia. 
In  sensory  aphasia,  the  patient  writes  paragraphically,  i.  e.,  confusing  the 
letters,  as  he  does  in  speech,  whereas  reading  is  more  affected  than  in  motor 
aphasia.  Alexia  persists,  more  frequently  only  severe  dyslexia.  Reading 
aloud  is  paralectic,  as  speaking  is  paraphasic,  and  even  the  internal  compre- 
hension of  speech  is,  for  the  most  part,  seriously  disturbed. 

The  clinical  picture  of  sensory  aphasia,  is  therefore,  as  follows: — Ces- 
sation of  speech  understanding,  or  serious  disturbance. 

Spontaneous  speech:  paraphasic  in  varying  degrees,  with  comparative 
wealth  of  syllables,  often  indeed  of  words. 

Imitative  speech :  cessation  or  serious  paraphasia. 

Writing:  paragraphic  (spontaneous  and  dictated). 

Copying:  retained. 

Reading  aloud:  paralexia. 

Understanding  of  reading:  seriously  affected. 

The  main  differences,  then,  between  motor  and  sensory  aphasia  are: 

Motor  Aphasia  Sensory  Aphasia 

Speech:  Lost.  Abundant,  but  paraphasic. 

Understanding:  Retained.  Lost. 

Understanding  Slightly  Seriously  disturbed, 

of  reading:  disturbed. 

Now,  in  sensory  aphasia,  word-deafness  commonly  departs  after  some  months 
(by  substitution  of  the  right  hemisphere).     By  strict  tests,  however,  defects 


TOTAL  APHASIA  529 

in  the  understanding  of  words  may  be  found  years  later.  Paraphasia,  para- 
graphia and  paralexia  are  usually  more  lasting.  Therefore  one  may  not 
always  expect  a  high  degree  of  word-deafness  from  an  old  injury  in  the  left 
temporal  lobe. 

While,  from  mere  destruction  of  the  left  temporal  speech  region,  not  loss 
of  speech,  but  paraphasia  results,  an  additional  focal  injury  in  the  right 
temporal  lobe  may  cause  word-deafness.  This  word-deafness  happens  in  an 
entirely  different  manner  from  that  of  motor  aphasia:  all  stimuli  coming 
from  the  outside  have  been  withdrawn  from  the  motor- word  center.  In  these 
two-sided  focal  injuries  in  the  temporal  lobes,  the  word-deafness  remains 
stable,  and  should  these  focal  injuries  occur  in  certain  places,  complete 
cerebral  deafness  persists.  Practically,  therefore,  temporal  word-dumbness 
is  differentiated  from  motor  aphasia  by  its  concomitant  word-deafness,  even 
common  deafness.     (Cf.  also  pages  534  and  356.) 

The  word  dumbness  of  motor  aphasia  resulting  from  extensive  frontal 
focal  injury,  is  quite  lasting.  But  even  in  this  case,  after  considerable  time — 
though  in  many  individuals  never — a  certain  retrogression  may  take  place; 
after  some  months  when  the  focal  injuries  are  small,  or  when  there  are  favor- 
able compensating  conditions. 

First  substantives  and  separate  infinitives  return,  and  adjectives  without 
particles,  without  inflection  and  declension,  one  after  the  other.  (Agram- 
matism or  Telegram  speech,  i.  e.  Took  walk,  dizzy,  sick,  fall,  hospital — as 
the  narration  of  the  course  of  his  illness.) 

By  this  telegraphic  style,  the  speech  of  motor  aphasia,  after  a  certain 
retrogression,  is  distinguished  from  the  speech  of  the  sensory  aphasic,  which 
commands  the  inflexions  and  forms  of  speech,  uses  many  well-built  circum- 
locutory phrases,  but  can  not  find  concrete  nouns  and  verbs. 

Furthermore,  the  former  is  distinguished  by  the  continuing  difficulty  in 
articulation.  In  improvement  in  motor  aphasia,  imitative  speech  returns 
before  spontaneous  speech,  and  can  become  fairly  good,  though  spontaneous 
speech  long  remains  inadequate. 

6.  Total  Aphasia 

More  frequently  than  a  lesion  confined  to  the  frontal  or  temporal  speech 
region,  we  find,  as  a  result  of  the  arterial  distribution  bringing  all  the  blood, 
in  the  whole  region  of  speech,  through  the  Art.foss.  Sylvii,  lesions,  that  affect 
both  regions,  and  therefore  causing  total  or  almost  total  (motor  and  sensory) 
aphasia.  Following  the  retrogression  of  the  word-deafness,  one  sees,  years 
later,  a  clinical  picture,  in  which  the  symptoms  of  motor  aphasia  predominate. 
Word-dumbness  conceals  the  paraphasia;  the  disturbance  of  speech  under- 
standing is  no  longer  very  serious. 

Writing  and  reading,  since  a  and  m  are  affected,  are  very  poor.     Hence 

34 


530  DISEASES  OF  THE  BRAIN 

it  happens,  that  old  cases,  in  which  the  lesions  occur  in  both  speech  regions, 
are  often  classed  clinically  only  as  motor  aphasia.  In  these  cases,  disturb- 
ances in  speaking,  writing  and  reading,  are  particularly  stable. 

This  clinical  picture  in  its  entirety  of  motor  and  sensory  aphasia  was 
named  by  Wernicke  "  cortical."  He  assumed  that  the  cortex  was  the  seat 
of  the  mnemic  images  in  question,  and  that  only  the  loss  of  mnemic  images 
gives  rise  to  the  complete  symptoms  portrayed.  Naturally,  this  must  not  be 
interpreted  so  literally  as  to  mean  that  the  lesion  must  affect  only  the  cortex. 
This  seldom  happens;  usually,  not  only  the  cortex  but  also  the  underlying 
white  matter  is  destroyed. 

This,  however,  makes  no  change  in  the  clinical  picture,  since  the  loss  of 
the  projection  and  association  fibres  that  run  through  the  white  matter,  can 
cause  no  additional  disturbances,  when  once  the  center  itself  is  destroyed. 

7.  Insular  Aphasias 

Between  the  frontal  and  temporal  lobes  lies  the  insular  (Island  of  Reil) 
speech  region.  Focal  injuries  in  the  Island  of  Reil  give  rise  to  very  different 
clinical  pictures,  which  to  this  extent,  resemble  motor  aphasia,  in  that  they 
disturb  the  expressive  phase  of  speech.  But  on  the  other  hand,  they  injure 
the  understanding  of  it  only  under  circumstances  to  be  more  definitely 
described.     They  are: 

1.  Possibly  by  injuring  the  cortex  of  the  insula,  since,  as  we  have  men- 
tioned, the  front  part  of  the  insular  cortex  may  belong  to  the  motor  speech 
center. 

2.  When  they  lie  subcortically  between  the  cortex  and  the  lenticular 
nucleus,  by  cutting  through  essential  bundles  of  white  fibres,  which  connect 
a  with  m,  namely,  the  external  capsule  and  the  extreme  capsule.  This 
interruption  of  part  of  the  paths  a — m  causes  difficulty  in  finding  words, 
paraphasia,  and  injures  imitative  speech,  but  that  less  than  spontaneous 
speech,  since  this  simplest  function  of  speech  is  naturally  capable  of  most 
resistance.  As  long  as  some  part  of  the  connections  a — m  are  retained — all 
of  which  by  no  means  pass  through  the  insula — imitative  speech  is  possible, 
if  not  wholly  intact.  This  is  directly  contrary  to  the  older  hypothesis,  which 
held  that  in  cases  of  insular  injuries,  it  is  precisely  imitative  speech  that  is 
the  most  seriously  disturbed.  Because  of  the  dissociation  of  a  and  m, 
written  speech  suffers  more  or  less,  since,  in  the  majority  of  people,  it  is 
dependent  upon  the  internal  word's  being  intact  (a — m). 

3.  Frequently  so-called  focal  insular  injuries  extend  into  the  white 
matter  of  the  third  frontal  convolution  and  the  Rolandic  operculum.  In 
this  case,  we  have  besides  the  breaks  (mentioned  under  2)  between  a  and  m: 
1.  a  break  in  the  left  centrifugal  projection  path  to  the  bulbar  cells;  2.  inter- 
ruptions in  the  fibres  that  pass  from  m  to  the  Rolandic  operculum;  3.  of  the 


PURE  WORD-DUMBNESS  531 

corpus  callosum  fibres;  4.  destruction  of  the  Fasc.  arcuatus  (cf.  page  471), 
another  very  important  connective  path  between  a  and  m. 

Hence,  a  break  between  a  and  m,  would,  on  the  one  hand,  be  more  vital 
than  in  two  (2);  on  the  other  hand,  a  subcortical  break  of  the  pathways 
leading  from  m  to  the  periphery  exists.  Therefore  one  finds  clinical  pictures 
closely  approximating  total  motor  aphasia:  very  poor,  almost  destroyed 
spontaneous  speech,  almost  no  imitative  speech,  and  disturbances  in  written 
speech.     Such  insular  focal  injuries  are  of  frequent  occurrence. 

4.  Often  the  focal  insular  injury  extends  posteriorly  down  into  the 
white  matter  of  the  temporal  lobe,  and  then  it  may  injure  the  understanding 
of  speech. 

From  this  it  is  clear,  that  focal  insular  injuries  may  have  different  effects, 
according  to  their  situation  and  extent;  they  all  cause,  however,  greater  or 
less  disturbance  in  the  expressive  phase  of  speech. 

8.  Pure  Word-dumbness 

Pure  Motor  Aphasia  ("Subcortical"  of  Wernicke) 

Besides  the  total  motor  aphasia  (cortical  of  Wernicke),  described  above, 
another  form  occurs,  but  more  seldom,  pure  word-dumbness  (subcortical 
form  of  Wernicke) .  In  this  form  only  expressive  speech,  that  is,  spontaneous 
and  imitative  (by  imitative  is  meant  ability  to  repeat  the  words  of  others), 
is  lost,  whereas  written  speech  (writing  and  reading)  is  retained.  In  some 
people,  as  has  been  mentioned  before  (page  524),  written  speech  is  more 
independent  of  motor- word  memories,  and  can  be  maintained  by  the  acoustic 
word  alone.  In  this  way,  the  rarer  cases,  in  which  the  lesion  is  the  same, 
as  in  total  motor-aphasia,  and  yet  written  speech  is  retained,  are  to  be 
explained.  The  majority  of  people,  especially  of  the  uneducated  have  to 
spell  the  word  internally  in  writing  (and  reading),  others  (especially  those 
well  versed  in  writing  and  reading),  need  the  motor  word  less  frequently  or 
not  at  all.  The  latter,  in  case  of  cortical  focal  injury  in  the  anterior  speech- 
region  will  show  pure  word-dumbness. 

This  would  be  pure  word-dumbness  by  destruction  of  the  motor  speech 
center  (first  form  of  pure  word-dumbness). 

In  the  majority  of  people  the  clinical  picture  of  pure  word-dumbness 
is  brought  about  by  the  fact  that  the  motor  center  for  speech  is  altogether 
or  largely  retained,  but  cut  off  from  the  operative  muscular  apparatus  of 
speech.  Under  such  conditions  the  motor  images  are  spared,  and  remain 
at  the  service  of  the  acts  of  reading  and  writing,  but  the  escape  of  stimuli 
into  the  bulbar  cells  is  prevented.  In  this  case  internal  speech  is  retained, 
the  memory  not  impaired,  so  that  we  dare  scarcely  call  it  aphasia.  But 
since  there  is  as  yet  no  question  of  an  injury  in  the  outgoing  neuro-muscular 


532 


DISEASES  OF  THE  BRAIN 


speech  apparatus,  the  disturbance  belongs  in  no  case  to  anarthria,  but,  as  a 
break  between  the  memory  apparatus  and  the  projection  apparatus,  lies 
between  the  two.  Now,  since  the  "purity"  of  this  word-dumbness  is 
always  only  approximate,  and  as  a  slight  admixture  of  disturbances  of 
internal  speech  can  almost  always  be  proved  present,  these  cases  are  usually 
and  justly  classified  under  the  aphasias. 

In  the  belief,  that  in  pure  word-dumbness,  the  cortical  part  of  the  center 
for  motor  images  is  retained,  and  only  the  white  matter  and  fibres  running 
to  the  speech  centers  are  interrupted,  Wernicke  called  this  form  "subcortical 
aphasia."  Since  the  older  hypothesis  of  a  direct  path  of  projection  (speech 
path)  from  the  motor  speech  center  to  the  bulbar  cells  has  become  doubtful, 
and  it  is  far  more  probable,  that  from  the  motor  speech  center,  the  stimuli 
reach  first  the  cortical  center  of  the  hypoglossal  and  facial  nerves  ("/*"  in 
the  scheme),  on  both  sides  in  the  inferior  third  of  the  frontal  convolution 
(Rolandic  operculum),  and  from  there  only  to  the  bulbar  cells,  one  should 
rather  think  of  a  break  in  these  connections  between  the  motor  speech 
center  and  both  sides  of  the  anterior  central  convolution,  which  can  actually 
be  caused  by  subcortical  focal  injuries  in  the  third  frontal  convolution  and 
the  adjacent  white  matter.  (Break  in  the  pathways  from  m  to  the  left  side 
of  the  Rolandic  operculum  and  in  the  corpus  callosum  paths  to  the  right  side 
of  the  Rolandic  operculum.) 

The  old  conception  of  subcortical  aphasia — better  replaced  by  pure  word- 
dumbness — may  not  find  anatomically  a  strict  application  in  words:  the 
meaning  is  that  a  break  in  the  paths  between  the  motor  speech  centers  and 
the  muscular  apparatus  of  speech  is  concerned.  Naturally  a  subcortical 
focal  injury7  outside  of  these  connective  paths,  can  interrupt  so  many  associa- 
tion fibres  of  the  motor  speech  center  with  the  whole  cortex,  that  the  motor 
speech  center  is  almost  isolated,  and  therefore  almost  as  funcrionless  as  if  it 
were  destroyed.  Such  a  subcortical  lesion  would  give  a  clinical  picture  of 
total  motor  aphasia.  On  the  other  hand,  a  subcortical  lesion,  according  to 
situation  and  extent,  may  be  of  such  a  nature,  as  to  spare  partly  the  fibres 
passing  from  the  speech  center  to  the  cortical  centers  of  hypoglossal  and 
facial  nerves  on  both  sides,  perhaps  the  commissural  fibres  on  the  right 
passing  through  the  corpus  callosum,  and  then  a  subcortical  focal  injury 
would  not  cause  even  pure  word-dumbness.  Finally  a  focal  injury  may 
destroy  part  of  the  cortex  without  wholly  incapacitating  it,  but  with  an  exten- 
sion into  the  white  matter,  break  the  connections  with  both  sides  of  the 
Rolandic  operculum.  Then  this  focal  injury,  partly  cortical  and  partly 
subcortical,  would  have  a  similar  effect  as  a  pure  subcortical  break  of 
the  connections  named.  It  is  evident,  that  the  conceptions  of  cortical  and 
subcortical  aphasia  are  not  anatomically  to  be  taken  literally,  but  mean  as 
much  as  incapacitation  of  the  cortical  speech  center  itself  and  a  mere  break 
between  this  and  its  subordinate  centers.     In  realitv,  manv  different  combina- 


PURE  WORD-DEAFNESS  533 

tions  of  lesions  may  form  one  or  the  other  clinical  picture.  But  it  has  been 
proved,  that  in  the  case  of  extensive  destruction  of  the  cortex  the  usual  result 
is  the  clinical  picture  of  total  motor  aphasia,  whereas  pure  word-dumbness 
results,  in  the  majority  of  cases  from  subcortical  lesions. 

Excluding  that  minority,  whose  written  speech  is  independent  of  the 
motor  speech  center,  we  may  say:  lesions,  that  spare  wholly,  or  partly  the 
cortex  of  the  frontal  speech  regions,  cause  only  the  loss  of  spoken  speech, 
and  spare  written  speech.  (Pure  word-dumbness,  second  form  of  pure 
word-dumbness.)  Those  lesions,  however,  that  to  a  great  extent  destroy  the 
cortex  of  the  frontal  speech  region,  usually  affect  written  as  well  as  spoken 
speech.  A  lesion,  moreover,  that  breaks  the  left  cortical  bulbar  tract,  in 
addition  to  a  second  lesion  which  breaks  the  commissural  paths  between 
m  and  the  right  Rolandic  operculum,  likewise  causes  pure  word-dumbness. 

9.  Pure  Word-deafness 

Likewise  we  have,  beside  total  sensory  aphasia,  an  isolated  or  pure 
word-deafness  {Lichiheim's  Disease;  subcortical  sensory  aphasia). 

In  this  disease,  only  comprehension,  and  as  a  natural  consequence, 
imitative  speech  and  dictated  writing  are  lost,  whereas  speaking,  writing,  and 
reading  are  retained.  Paraphasia,  paragraphia,  and  paralexia  are  wanting 
to  make  this  total  sensory  aphasia.  That  a  focal  injury,  which  destroys 
the  sensory  speech  center,  causes  pure  word-deafness  (because  an  individual 
independence  of  spoken  and  written  speech  of  auditory  images  is  in  question, 
corresponding  to  the  oft  discussed  relation  when  motor- word  images  are 
disturbed)  is  necessarily  a  thing  of  rare  occurrence. 

Even  the  dependence  of  written  speech  on  the  acoustic  word  has  fewer 
exceptions  than  that  on  the  motor-word.  In  a  case  of  pure  word-deafness, 
one  must  think  of  the  persistence  of  the  substratum  of  auditory  word  images, 
and  of  mere  exclusion  of  these  from  acoustic  stimuli.  This,  as  Lichtheim 
has  taught,  is  caused  in  the  first  instance,  by  a  subcortical  focal  injury  in 
the  left  temporal  lobe,  situated  so  as  to  break  the  incoming  of  all  acoustic 
stimuli  to  the  left  temporal  lobe,  and  possibly,  too,  the  corpus  callosum 
connection  between  the  hearing  center  of  the  right  and  that  of  the  left  hemi- 
sphere. Then  the  possession  of  auditory  word  images  is  established  by  the 
fact  that  reading,  speaking,  and  writing  are  intact,  whereas  the  exclusion  of 
these  auditory  word  images  from  all  acoustic  stimuli,  causes  the  loss  of 
speech-comprehension.  A  second  possibility  as  to  the  establishment  of  pure 
word-deafness  occurs,  if  with  Flechsig,  one  separates  the  acoustic  projection 
center  sharply  from  the  mnemic  acoustic  center  (cf.  Fig.  131).  Then,  the 
destruction  of  this  center  (i.  e.,  particularly  of  the  transverse  convolution 
in  the  temporal  lobe)  deprives  the  left  hemisphere  of  acoustic  stimuli  and 
hence  cuts  off  auditory  word  images  from  the  periphery,  whereas  they  could 


534  DISEASES  OF  THE  BRAIN 

still  develop  their  inter  cortical  effectiveness  in  speaking,  writing,  and  reading. 
This  would  be  pure  word-deafness  due  to  cortical  focal  injury  (in  the  trans- 
verse convolution).  It  is  doubtful  whether  pure  word-deafness  could  arise 
from  a  break  in  a  path  alpha — a,  since  it  is  doubtful,  whether  alpha  and  a  are 
connected  by  a  complete  set  of  association  fibres. 

Pure  word-deafness  persists,  provided  the  right  temporal  lobe  does  not, 
in  the  course  of  time,  also  take  the  place  of  the  left,  which,  in  the  case  of 
subcortical  focal  injuries,  often  fails  to  happen.  Here,  too,  as  in  total  sensory 
aphasia,  the  comprehension  of  the  spoken  word  is  already  lost.  Tones  are 
heard,  but  only  as  a  strange  confused  noise.  If  the  comprehension  of  the 
spokm-wordis  lost,  naturally  the  meaning  of  the  word  is  not  grasped,  i.  e.,  the 
comprehension  of  the  meaning  of  the  word  is  wholly  lost. 

To  establish  a  case  of  pure  word-deafness,  it  must  be  proved  that  hearing 
is  possible.  If  hearing  is  seriously  impaired  on  both  sides  by  disease  of  the 
labyrinths  or  of  the  auditory  tracts  or  centers,  the  comprehension  of  speech 
may  be  lost  as  a  result  of  defective  hearing,  alone.  Then  we  have  pseudo- 
speech-deafness,  a  mere  consequence  of  defective  hearing.  Since  Bezold 
proved,  that  total  loss  of  the  tone  range  b'  to  g",  or  serious  weakening  within 
this  range,  eo  ipso,  destroys  comprehension  of  speech,  one  must  prove  not 
only  the  continuous  range  of  tones  but  also  that  the  patient  hears  with  suf- 
ficient clearness  within  the  range  of  the  tones  concerned.  It  is  not  suf- 
ficient to  prove  the  existence  of  hearing,  by  received  perception  of  noises, 
whistling,  clapping  of  hands,  ringing,  etc.,  since  if  the  tone-range  mentioned 
above  is  lost,  speech  will  not  be  understood,  even  if  the  hearing  of  the  other 
tones  of  the  entire  range  of  tones  is  intact.  Furthermore,  to  distinguish 
subcortical  sensory  aphasia  from  loss  of  comprehension  of  speech,  due  to  gen- 
eral deafness,  this  fact  serves — that  the  former  is  never  absolutely  pure,  but 
that  separate  paraphasic  or  paragraphic  symptoms  prove  that  it  is  not  a 
question  of  mere  defect  in  hearing. 

There  is  besides  a  still  rarer  form  of  speech-deafness,  in  which  letters, 
syllables,  and  short  words  can  be  comprehended  and  repeated,  but  larger 
words  and  sentences  are  not  understood,  not  even  in  the  sound  of  the  words. 
With  this  there  is  only  very  slight  paraphasia,  paragraphia  and  paralexia,  so 
that  this  form  (because  of  the  small  proportion  of  disturbances  in  internal 
speech)  approximates  pure  word-deafness.  A  cortical  focal  injury  in  the 
speech  center,  which  injures  auditory  word  images  less,  than  their  communi- 
cation with  the  acoustic  stimuli,  might  be  the  cause. 

10.  Transcortical  Aphasias 

(Including  Amnesic  and  Visual  Aphasia) 

By  transcortical  aphasias,  Wernicke,  indorsing  the  theory  and  classifica- 
tion of  Lichtheim,  understands  those,  in  which  the  motor  and  sensory  speech 


TRANSCORTICAL  APHASIA 


535 


at 


-■2 


--o 


Mouth 

Wernicke's 
scheme,     i,  Cortical  sen- 


Ear 
Fig.  154.- 


centers  themselves,  and  their  connection  with  each  other  and  with  the  per- 
iphery are  retained,  but  one  of  the  two  is  cut  off  from  the  perception  area, 
i.  e.,  from  all  the  rest  of  the  cortex.  Psychologically,  the  word  (a — m)  is 
intact,  but  one  of  its  components  is  cut  off  fromB.  The  complex  ear  (a — m) 
mouth  (Fig.  154)  is  therefore  free. 

W.  thought  this  was  caused  by  a  break  in  the  association  fibres  which 
converge  from  the  entire  cortex  to  one  of  both  speech  centers. 

In  his  scheme,  disturbance  in  a  and  m  means  cortical  aphasia,  a  break  in 
the  pathway  between  m  and  a,  subcortical  aphasia,  and  a  break  in  the  paths 
from  B  to  m  or  to  a,  transcortical  aphasia  (correspondingly  motor  or  sensory). 

Clinically  this  is  marked  especially,  by  the  ability  to  repeat  words  (the 
comprehension  of  spoken  speech  is  retained) ,  inasmuch 
as  the  section  ear — a — m — mouth  is  free,  but  that  either 
the  word  can  not  be  found  from  the  meaning  (transcor- 
tical motor  aphasia,  6)  or  that  the  meaning  of  the  word 
heard  cannot  be  found  (loss  of  comprehension  of 
word  meanings)  transcortical  sensory  aphasia  (3). 
The  disturbances  in  writing  and  reading  ascribed  to  the 
transcortical  forms  (spoken  speech,  dictated  writing 
retained)  are  realized  only  approximately.  The  clin- 
ical reality  of  these  transcortical  forms,  with  this  limi- 
tation, is  certain:  we  often  have  patients,  who  can  re-  sorv  aphasi£i 
peat  words  (sound  comprehension  retained),  but  who    tical  sensory  aphasia.    3, 

,  .  .     n  ...      ,  ,  Transcortical     sensory 

do  not  understand  them  readily  (meaning  comprehen-  aphasia.  4,  Cortical  mo- 
sion  lost)  or  have  difficultv  in  finding  words  from  their    tor  ap^ia-    5,  Subcor- 

'  .  tical   motor   aphasia,     o, 

meaning.     Often  these  Pwo  are  associated :  difficulty  in    Transcortical  motor  apha- 
the  comprehension  of  the  meaning  of  words  and  very   ^  (^^1^° not a con- 
great    difficulty  in    finding   words,    though   imitative    firmed). 
speech  is  retained. 

But  anatomically,  the  relations  assumed  by  Wernicke  are  not  to  be 
realized  in  the  simple  manner,  taken  from  the  brain,  as  he  supposed.  It  is 
not  merely  the  case  that,  when  imitative  speech  is  lost,  the  focal  injury  is 
in  the  center,  and  when  it  is  retained,  that  the  focal  injury  lies  in 
the  tracts  converging  to  the  center.  The  principle,  in  Wernicke's  suppo- 
sition, that  when  imitative  speech  is  lost,  the  real  conveyors  of  the  motor  or 
sensory  word  are  destroyed,  and  that  when  it  is  retained,  they  are  retained, 
but  can  not  be  reached  from  the  superior  cortical  seat  of  perception,  holds 
good  in  part,  but  under  very  different  anatomical  conditions,  and  sometimes 
the  focal  injuries  are  so  situated  that  one  does  not,  without  further  difficulty, 
see  this  effect.  The  empirical  conditions,  and  the  knowledge  that  the  direct 
path  B — m  is  of  no  great  importance,  that  moreover,  spontaneous  speech 
mainly  uses  the  same  pathway  as  imitative  speech,  namely  the  path  a — m, 
necessitate  a  modification  of  this  part  of  Wernicke's  view.     Retention  and 


536  DISEASES  OF  THE  BRAIN 

loss  of  imitative  speech  depend  on  other  conditions  beyond  the  situation  of 
the  focal  injuries,  that  is,  on  the  degree  of  lesion.  Imitative  speech,  naturally, 
is,,  on  the  whole,  that  function  of  speech,  most  capable  of  resistance,  so  that 
slighter  lesions  of  the  same  region,  the  destruction  of  which  causes  total 
aphasia,  permit  of  some  imitative  speech.  This  is  true  also  of  the  paths  that 
lead  to  m  (a-m).  In  partial  lesions,  the  powerful  stimuli,  which  come  from 
the  periphery,  are  allowed  to  pass,  while  the  weaker  spontaneous  stimuli 
coming  from  B  are  not. 

The  word  transcortical  includes  a  clinico-symptomatological,  an  anatom- 
ical' and  a  psychological  set  of  conditions,  all  three  of  which  are  not  always 
fulfilled  together.  We  shall  use  the  word  here  only  in  its  clinico-symptomato- 
logical sense,  i.  e.,  consider  as  transcortical  those  forms  only  in  which  imita- 
tive speech  is  retained. 

This  is  not  the  place  to  discuss  the  value  of  the  other  meanings  of  the 
word  transcortical. 

(A)  Transcortical  motor  aphasia  is  here,  the  loss  or  serious  disturbance  of 
spontaneous  speech,  associated  with  retention  of  imitative  speech. 

This  is  found  in: 

I.  Slight  injury  of  m  itself,  which  destroys  the  possibility  of  stimulating 
the  center  of  perception  from  the  periphery  (via  a),  without  injuring  those 
fibres  in  the  center  that  convey  the  motor-word  to  such  an  extent  that  they 
can  not  be  innervated  by  the  stronger  stimulus  of  the  spoken  word. 

II.  Slight  injury  of  the  paths  from  a  to  m,  a  case  already  mentioned  under 
focal  insular  injuries  (page  530)  and  of  the  accessory  paths  from  B  to  m  from 
the  same  cause. 

III.  When  one  has  to  consider  the  possibility,  that  the  return  of  imitative 
speech  has  become  a  function  of  the  right  hemisphere. 

In  cases  1  to  3,  imitative  speech  is  better  than  spontaneous  speech,  but 
nevertheless  very  much  impaired  and  faulty. 

IV.  The  requirement  of  the  retention  of  imitative  speech  is  more  strictly 
complied  with  in  so-called  amnesic  aphasia  or  verbal  amnesia,  in  which,  it  is 
true,  even  spontaneous  speech  is  not  wholly  lost.  It  is  clinically,  and  prin- 
cipally, a  slighter  form  of  transcortical  motor  aphasia,  though  the  mode  of 
its  origin  be  other  than  the  one  accepted  by  Wernicke  for  the  latter  case. 

There  is  great  difficulty  in  finding  words,  but  if  the  word  is  suggested, 
it  is  immediately  recognized  as  the  right  one,  and  repeated  with  ease  and 
correctness.  In  this  verbal  amnesia,  it  is  most  difficult  to  find  nouns  and 
verbs  for  concrete  objects  or  actions,  whereas  in  contrast  to  forms  1  and  2, 
abstract  nouns  and  inflected  parts  of  speech,  particles,  prepositions,  etc.,  as 
well  as  inflections  and  declensions  are  retained.  This  form  is  found  under 
manifold  conditions,  always  when  one  of  the  stations  by  which  the  process  of 
speech  must  pass,  before  reaching  the  motor  center,  is  slightly  injured. 
Therefore : 


VISUAL  APHASIA  537 

1.  When  the  sensory  (temporal)  speech  center  is  but  very  slightly  injured 
(say,  by  moderate  atrophy).  This  small  injury  does  not  destroy  imitative 
speech  and  comprehension,  but  makes  difficult  the  wakening  of  auditory  word 
images  from  the  perception  of  them  (the  function  most  easily  disturbed) . 

2.  When  the  connections  between  the  sensory  speech  center  (a)  and  B  are 
slightly  injured,  because  of  which  it  is  difficult  to  find  words,  while  the 
meaning  of  the  word  heard  is  still  comprehended. 

3.  When  the  perceptive -region  itself,  that  is,  the  entire  cortex  is  injured, 
even  before  the  perception  is  so  seriously  disturbed,  that  verbal  amnesia  is 
lost  in  imbecility,  the  pathways  to  the  sensory  speech  center  are,  in  their  very 
beginnings,  injured. 

In  diffuse  processes  (paralysis,  senile  atrophy,  arterio-sclerosis)  frequently 
several  of  the  conditions  named  under  1  to  3  are  fulfilled,  so  that  in  those . 
cases,  amnesic  aphasia  is  quite  usual. 

But,  moreover,  focal  injuries  back  of  a,  which  are  not  sufficiently  extended, 
to  produce  a  case  of  transcortical  sensory  aphasia  (to  be  discussed  later), 
reduce  spontaneous  speech  to  the  lowest  degree,  without  in  the  least  affecting 
imitative  speech. 

The  so-called  visual  (optic)  aphasia  is  only  a  peculiar  form  of  amnesic 
aphasia.  According  to  its  definition,  objects  seen  can  not  be  named,  and 
those  objects,  that  we  know  exceedingly  well  and  mostly  by  sight,  can  not  be 
designated  in  free  speech.  On  the  other  hand,  objects  perceived  by  any  other 
sense,  for  instance,  that  of  touch,  it  should  be  possible  to  name.  In  reality, 
the  most  frequent  form  is  an  optic-tactile  aphasia,  i.  e.,  neither  by  sight,  nor 
by  touch,  is  the  name  of  an  object  found,  though  it  may  be  easily  found  by 
ear.  A  trumpet,  for  instance,  that  has  been  seen  and  touched,  can  not  be 
named,  whereas  as  soon  as  it  sounds,  the  name  is  immediately  suggested. 
In  this  visual  aphasia,  it  is  generally  an  extension  of  amnesic  aphasia,  that 
we  find,  in  which,  the  inability  to  name  objects  (in  contrast  to  common  parts 
of  speech  and  abstract  nouns)  is  more  than  usually  conspicuous,  in  which 
the  perception  of  the  object  creates  less  favorable  conditions  for  naming  it, 
than  exist  in  free  speech,  and  naming  by  acoustic  characteristics  proves  to  be 
easier. 

This  hall-mark  of  amnesic  aphasia  is  found  especially  in  cases  of  abscesses 
in  the  temporal  lobes,  which  mostly  lie  at  the  base  of  the  temporal-occipital 
lobes,  furthermore  in  other  injuries  in  the  connecting  pathways  between  the 
temporal  and  occipital  lobes,  even  in  the  occipital  lobes  themselves.  They 
have  this  in  common,  namely,  that  they  interrupt  connective  paths  between 
a  and  the  occipital  lobe,  the  seat  of  the  essential  optic  components  of 
perception. 

Amnesic  and  especially  optic  aphasia  is  transcortical,  even  in  that  meaning 
of  the  word  that  has  been  heretofore  neglected:  the  hindrance  in  speech  does 
not  lie  in  the  word  centers  themselves  (a  or  m),  nor  in  their  connections  with 


538  DISEASES  OF  THE  BRAIN 

one  another  and  the  periphery,  but  beyond  the  centers,  on  the  path  that  must 
be  transversed  by  the  stimulus  before  it  can  reach  the  word  centers  from 
preception. 

Since  a  is  for  us  an  intermediate  station  for  the  stimulus  from  C  to  m,  in 
this  sense  every  hindrance  in  expressive  speech  caused  by  a  lesion  in  a  is 
transcortical,  especially  for  instance,  the  word-dumbness  due  to  bilateral  in- 
juries in  the  temporal  lobes  (see  page  529).  In  this  case,  however,  imitative 
speech  also  is  lost  so  that  the  distinctive  feature  that  clinically  and  particularly 
characterizes  transcortical  aphasias,  is  absent.  This  difficulty  we  may  only 
point  out  here. 

(B)  Transcortical  Sensory  Aphasia. — We  have  seen  that  an  injury  to 
the  connective  pathway  between  a  and  B,  that  is,  a  partial  interruption  only, 
causes  transcortical  motor  aphasia,  seriously  hindering  spontaneous  speech 
(that  is  the  stimulus  in  the  direction  B  to  a),  but  does  not  destroy  the  pathway 
in  the  opposite  direction  a  to  B :  the  connection  of  the  percept  with  the  word, 
the  comprehension  of  the  meaning  of  the  word. 

If,  however,  a  total  interruption  occurs  in  the  paths  from  B  to  a,  as  op- 
posed to  partial  interruption,  or  if  B  itself,  is  seriously  injured  (asymbolia  in 
the  older  sense  of  the  word)  we  have  transcortical  sensory  aphasia:  imitative 
speech  is  retained,  often  occurring  irrepressibly  (echolalia),  but  comprehen- 
sion of  what  is  spoken  is  lost.  This  happens,  because  no  concept  joins  the 
auditory  word  image  evoked  in  a,  but  the  stimulus,  to  which  the  path  to  B  is 
closed,  flows  out  into  the  free  path  a  to  m  of  imitative  speech.  Anatomically 
transcortical  sensory  aphasia  is  shown  frequently  by  marked  atrophy  in  the 
neighborhood  of  the  first  temporal  convolution,  that  is,  in  the  second  and  third 
temporal  convolution  and  even  further  back  (marked  atrophy  in  the  white 
matter  of  the  temporal  lobe).  It  occurs  often  side  by  side  with  general 
atrophy  and  corresponding  injury  in  the  perception  centers.  (Asymbolic 
disturbances  in  the  older  sense.)  Several  focal  injuries,  moreover,  in  the 
posterior  half  of  the  brain  can  bring  about  this  isolation  of  a,  which  spares 
only  the  connection  with  m. 

Now,  it  must  be  remarked,  that,  if  only  a  partial  interruption  in  a — B, 
brought  about  almost  complete  loss  of  expressive  speech,  a  greater  interrup- 
tion would  certainly  alter  expressive  speech  in  a  considerable  degree.  As  a 
matter  of  fact,  we  have  almost  always  associated  with  transcortical  sensory 
aphasia  a  serious  disturbance  in  spontaneous  speech.  Hence  transcortical 
sensory  aphasia,  as  a  rule,  is  associated  with  transcortical  motor  aphasia; 
only  the  sound  of  the  word  is  comprehended,  and  parrot-like  imitated,  but 
neither  is  the  meaning  understood,  nor  spontaneous  speech  to  any  extent 
possible.  A  complete  restriction  to  the  symptom-complex  actually  found  in 
transcortical  sensory  aphasia  (i.  e.,  imitative  speech,  but  lack  of  compre- 
hension in  the  meager  spontaneous  speech)  is  possible  only  in  a  person  in 
whom,  exceptionally,  the  pathway  B — m  is  particularly  efficient. 


SENSORY  APHASIA  539 

Summary  of  Temporal  Lesions. — To  summarize  what  has  been  said 
about  sensory  aphasic  disturbances,  an  interruption  of  the  path  leading  to 
the  left  auditory  center  (including  the  corpus  callosum  connection),  as  well 
as  one  in  the  left  auditory  center  itself  (middle  third  of  the  superior  temporal 
convolution  and  transverse  convolution),  creates  pure  word-deafness.  A 
destruction  of  the  "sensory  speech  center"  (posterior  third  of  the  superior 
temporal  convolution  and  that  part  of  the  supra  marginal  gyrus,  lying 
directly  behind  it)  causes  word  deafness  combined  with  paraphasia,  para- 
graphia and  paralexia.  Where  there  is  deafness  to  the  word-saund,  we  find, 
in  consequence  deafness  to  the  word-meaning. 

A  total  isolation — with  the  exception  of  the  connection  with  m — of  the 
sensory  speech  (serious  atrophy  of  the  whole  temporal  lobe,  extensive  focal 
injuries  in  the  depths  back  of  and  around  the  sensory  speech  center),  destroys 
only  the  comprehension  of  the  meaning  and  not  that  of  the  sound  of  the  word, 
which  depends  on  imitative  speech,  and  at  the  same  time  produces  the 
greatest  difficulty  in  finding  words.  But  if  the  sensory  speech  center  or  its 
connection  with  B  is  but  slightly  or  partially  disturbed,  only  amnesic  aphasia 
results  (in  the  second  case,  especially,  optic  aphasia),  whereas  compre- 
hension of  word  sound  and  meaning  are  retained. 

It  is  now  self-evident,  and  this  is  true  of  all  cerebral  lesions,  that  diseases 
affecting  the  left  temporal  lobe,  whether  they  be  neoplasms  or  focal  injuries 
of  vascular  origin,  only  exceptionally,  restrict  their  destructive  and  far  reach- 
ing consequences  to  the  substratum  of  one  element  or  an  element  connective, 
as  sharply  differentiated  in  the  scheme.  According  to  their  various  situa- 
tions and  extent,  and  to  the  participation  of  many  pathways,  represented  by  a 
single  line  in  the  scheme,  various  combinations  of  symptoms  appear.  Loss  of 
comprehension  of  word-sound  and  word-meaning,  difficulty  in  finding  words 
and  paraphasia,  disturbances  in  writing  and  reading,  combined  in  varying 
degrees,  form  the  typical  clinical  pictures  of  the  diseases  which  can  be  classi- 
fied only  approximately  according  to  theoretically  developed  forms.  Pure 
word-deafness,  total  sensory  aphasia,  and  "transcortical"  sensory  aphasia, 
are  only  three  particularly  striking  types  of  sensory  aphasia,  the  diagnosis 
of  which  has  great  instructive  value. 

In  order  to  comprehend  all  real  differences,  one  would,  in  each  case 
have  to  take  into  account,  the  extent  to  which  the  cortex,  the  projection — 
association — and  commissural  fibres  are  affected,  and  would  moreover,  have 
to  know  clearly  the  function  of  each  of  them,  a  knowledge  which  we  have 
unfortunately  by  no  means  attained. 

Disturbances  in  Written  Speech 
{a)  Alexia  and  Agraphia;  (b)  Pure  Alexia;  (c)  Pure  Agraphia 
We  noticed  that  written  speech  was  usually  affected  with  spoken  speech: 
writing,  seriously  in  both  main  forms  of  aphasia;  reading,  seriously  in  sensory, 


54° 


DISEASES  OF  THE  BRAIN 


less  seriously  in  motor  aphasia.     Only  in  the  so-called  pure  forms  was  written 
speech  retained  intact. 

There  are  disturbances  in  written  speech,  that  are  almost  isolated,  i.  e., 
reading  and  writing  are  seriously  impaired,  while  little  harm  is  done  to  the 
other  functions  of  speech. 

Focal  injuries,  situated  directly  behind  the  sensory  speech  center,  that  is, 
in  the  angular  gyrus  and  the  upper  layers  of  its  white  matter,  cause  agraphia 
and  alexia,  associated  with  but  slight  symptoms  of  paraphasia,  and  difficulty 
in  finding  words.     Those  focal  injuries,  however,  which  from  the  medial 

surface  of  the  center  extend  for- 
wards into  the  deep  white  matter 
of  the  angular  gyrus,  cause, 
especially,  pure  alexia,  i.  e.  writ- 
ing is  retained,  reading  lost. 
With  this  right  hemianopia  is 
almost  always  associated, 
whereas  superficial  focal  in- 
juries in  the  angular  gyrus, 
which  cause  the  loss  of  both 
reading  and  writing,  often  do 
not  produce  hemianopia. 

The  simplest  explanation  of 
these  statements,  is  as  follows : 

The  optic  radiation  runs 
through  the  deep  white  matter 
of  the  angular  gyrus,  outside  of 
the  posterior  horn  through  the 
white  matter  of  the  occipital 
lobe,  to  the  calcarine  region, 
the  left  and  right  visual  center 
(s  and  s').  From  here  pass  connecting  fibres  (not  shown  in  Fig.  155)  to  the  con- 
vexity of  the  occipital  lobe,  the  seat  of  visual  images  (form  memories)  (s — o). 
These  are  registered  mostly  in  the  left,  but  also  in  the  right  occipital  lobe.  The 
connective  fibres  from  the  right  occipital  lobe  pass  through  the  splenium 
to  the  convexity  of  the  left  occipital  lobe  (s' — o).  From  the  sensory  speech 
center  an  association  path  passes  to  the  convexity  of  the  occipital  lobe  (a — 0) . 
From  o  to  the  hand  center  (gr)  passes  the  path  by  which  visual  directions 
are  sent  to  the  hand  (Fig.  155). 

Now,  if  a  focal  injury  destroys .  (1)  the  angular  gyrus,  starting  from  the 
convexity,  it  interrupts  the  path  a — o.  The  consequence  is,  that  the  sound 
pictures  of  the  letters  aroused  in  a,  do  not  awaken  the  corresponding  visual 
images  in  o,  which  is  necessary  for  writing;  whence  agraphia.  In  reading, 
the  opposite  takes  place;  the  stimuli  of  both  fields  of  light  are  not  carried 


Fig.  155. —  (Explanation  in  the  text.)  Focus  1 
causes  alexia  and  agraphia.  Focus  2  pure  alexia  with 
hemianopia.  Focus  3  pure  agraphia  of  the  right  hand 
only.    Focus  4  pure  bilateral  agraphia. 


ALEXIA:  AGRAPHIA  541 

over  to  a,  so  that  images  of  letters  fail  to  call  up  their  sounds  (for  the  sake 
of  simplicity,  we  pay  no  attention  to  the  secondary  connective  path  0 — m), 
and  what  is  seen,  is  not  understood;  whence  alexia.  Because  of  the  inter- 
ruption in  an  important  path  from  o  to  a,  there  results  difficulty  in  finding 
words,  and  because  of  the  proximity  of  the  focal  injury  to  a,  there  is  fre- 
quently slight  paraphasia.  But  if  the  focal  injury  lies  in  2,  it  interrupts, 
on  the  one  side,  the  optic  radiation,  hence  right  hemianopia,  on  the  other, 
the  commissural  paths  from  the  right  occipital  lobe  (s' — 0)  so  that  no  optic 
stimulus  reaches  the  left  o.  The  visual  stimuli,  that  reach  only  the  right 
occipital  lobe,  do  not  reach  a,  and  therefore  call  up  no  auditory  images,  so 
reading  is  lost.  But  focal  injury  in  2  does  not  break  the  connections  of  a 
and  m  with  o,  nor  those  between  o  and  gr,  so  that  there  is  no  reason  for  the 
loss  of  writing,  hence  pure  alexia. 

If  a  focal  injury  (3  and  4)  interrupts  the  path  from  the  left  occipital  lobe 
to  the  center  for  the  right  (or  the  left)  hand,  pure  agraphia  appears,  usually 
in  both  hands,  but  under  certain  conditions,  only  in  the  right.  Pure  agraphia 
is  also  often  a  partial  symptom  of  apraxia  of  the  hand  affected. 

If  one  considers,  that  in  the  brain,  it  is  not,  as  in  the  scheme,  a  matter 
of  linear  connection,  but  of  numerous  and  various  connections  of  fibres, 
that  these  pass  to  the  other  categories  of  fibres,  on  various  levels  and  in 
various  situations,  that  the  size,  extent,  direction,  form,  and  number  of  the 
focal  injuries  create  the  most  varied  combinations  of  interruptions,  it  is 
then  clear,  that  the  above  linear  scheme  is  merely  a  simple  chart,  but  that, 
in  reality,  manifold  combinations  and  varieties  may  appear.  Suppose  for 
instance,  that  focal  injury  2  extends  an  outshoot  so  far  laterally,  that  o — gr,  too, 
is  interrupted,  then  we  shall  have  alexia  associated  with  agraphia. 

Pure  alexia  is  caused  by  a  focal  injury  in  the  region  supplied  by  the 
posterior  cerebral  artery. 

(b)  Agnostic  Disturbances 

Word-deafness  and  reading  blindness  are  really  special  manifestations 
of  disturbances  of  cognition.  The  patient  in  reading  blindness  sees,  but 
fails  to  understand  what  is  seen.  Perception  is  retained,  but  comprehension 
lost.  Reading  blindness  is  agnosia  for  written  letters,  word-deafness 
agnosia  for  word-sounds.  In  these  receptive  aphasic  disturbances,  it  is  a 
question  of  agnosia  for  conventional  signs,  for  the  symbols  of  speech. 
Speaking  of  agnosia,  in  the  stricter  sense,  one  means  corresponding  dis- 
turbances for  the  things  of  the  outer  world,  namely,  for  all  sense  impressions, 
that  are  not  symbols.  (Object  agnosia.)  If  say,  a  comb  is  seen,  but  its 
significance  not  recognized,  because  the  complex  of  visual  perceptions  does 
not  awaken  those  associations  that  normally  appear,  for  instance,  that  the 
comb  is  the  thing  with  which  we  comb  our  hair,  that  it  belongs  with  the 
brush,  etc.,  an  agnostic  disturbance  is  present.     It  is  not  a  matter  of  making 


542  DISEASES  OF  THE  BRAIN 

the  connection  of  the  symbol  and  its  import,  but  of  the  object's  real  bearing, 
origin,  purpose,  etc.  According  to  the  sense  affected,  one  differentiates 
between  acoustic,  visual,  tactile,  gustatory  and  olfactory  agnosia. 

i.  Acoustic  agnosia  or  mind  deafness  is  characterized  by  the  lack  of 
comprehension  of  speech  sounds  and  all  possible  auditory  stimuli,  such  as 
cries  of  animals,  musical  instruments,  cracking  of  the  whip,  clinking  of 
money,  etc.  It  occurs  associated  with  sensory  aphasia,  when  the  focal 
injury  is  in  the  left  temporal  lobe. 

2.  Visual  agnosia  or  mind  blindness.  The  patient  can  discuss  the 
form  and  even  the  color  of  objects,  but  they  are  strange  to  him;  he  not  only 
can  not  name  them,  when  shown,  as  in  visual  aphasia,  but  can  really  not 
recognize  them. 

Mental  blindness  results  from  lesions  in  the  occipital  lobes  and  generally 
occurs  when  the  focal  injuries  are  bilateral.  Usually  by  a  lesion  in  the  visual 
center,  or  optic  radiation,  half  or  at  least  a  quarter  of  the  field  of  vision  is 
excluded,  i.  e.,  for  this  part  of  the  visual  field  the  person  is,  perceptively 
speaking,  blind.  The  comprehension  of  the  impressions  received  by  the 
unaffected  parts  of  the  field  of  vision,  is  lost  because  of  an  interruption  in  the 
association  fibres,  which  run  from  the  visual  center  to  the  seat  of  visual  form 
memories  in  the  convexity  of  the  occipital  lobe,  or  because  of  the  destruction 
of  the  center  itself  or  its  connections  with  the  other  cortical  areas.  This  can 
be  caused  by  various  combinations  of  injuries,  which  affect  the  white  matter 
and  the  convexity  of  the  occipital  lobe,  and  those  parts  of  the  parietal  lobe 
lying  directly  in  front  of  it.  One  of  both  injuries  must  always  be  on  the  left; 
focal  injuries  to  the  right  occipital  lobe  do  not,  except  in  left-handed  people, 
cause  mental  blindness.  This  proves,  that  the  stability  and  delicacy  of 
visual  images  in  the  left  hemisphere  are  greater  than  those  in  the  right. 
Indeed,  in  the  majority  of  people  the  left  hemisphere  is  so  predominantly 
the  seat  of  visual  images,  that,  in  them,  an  injury  that  is  only  left-sided  effects 
a  considerable  degree  of  mental  blindness.  Mental  blindness  caused  only 
by  a  left-sided  injury,  occurs,  in  such  cases,  in  the  following  manner:  destruc- 
tion of  the  left  visual  center  or  of  the  optic  radiation  entirely  cuts  off  optic 
stimuli  from  the  right  hemisphere  (right  hemianopia).  The  focal  injury 
further  breaks  the  corpus  callosum  connection  between  the  right  occipital 
lobe  and  the  left  hemisphere  (splenium  or  forceps),  whereby  the  visual 
stimuli  reaching  the  right  field  of  vision,  cause  perceptions,  but  are  not 
understood,  since  they  do  not  reach  the  seat  of  memories  stored  up  predomi 
nantly  on  the  left  side.  At  the  same  time  there  naturally  occurs  pure  alexia, 
since  the  mechanism  of  mental  blindness,  due  to  one-sided  focal  injury, 
operates  as  in  pure  alexia.     Still  other  combinations  of  lesions  are  possible. 

Usually  perceptive  and  gnostic  disturbances  are  found  together,  so  that 
for  one  part  of  the  field  of  vision  there  is  blindness,  and  for  that  part  of  the 
field  that  has  been  retained,  mental  blindness. 


MIND  BLINDNESS  543 

As  a  rule,  the  acuity  of  vision  is  also  impaired,  often,  too,  there  is  some 
color  blindness.  Before  one  diagnoses  mental  blindness,  it  must,  of  course, 
be  proved  that  these  perceptive  disturbances  are  not  so  weighty,  that  it  is  not 
due  to  the  presentation  of  data,  insufficient  for  the  recognition  of  the  objects. 
(Pseudo-mental  blindness.) 

In  many  cases  of  mental  blindness  visual  memories  are  lost,  in  others, 
they  merely  can  not  be  aroused  by  centripetal  stimuli  but  the  patient  can  some- 
how, by  description,  or  drawing,  indicate  the  possession  of  memory. 

Or,  although  the  visual  memories  are  really  aroused,  the  associated  memo- 
ries of  the  other  senses,  tactile,  acoustic,  can  not  connect  with  them.  Often 
the  reason  of  failure  to  recognize  is  that  the  composition  of  the  concept  of  the 
object  from  the  various  sensory  data  is  impossible.  The  conceptions,  then, 
remain  a  chaos  of  simple  impressions,  which  do  not  flow  together  to  form 
concepts  of  concrete  objects. 

Usually,  not  only  form  memories,  but  also  color  memories  are  injured; 
the  patient  can  not  imagine  the  color  of  the  frog,  of  the  carriage,  of  the  blood. 
Occasionally  color  memories  are  retained;  but  it  is  an  exception  when  form 
memories  are  retained  and  only  color  memories  lost.  Usually,  in  mental 
blindness,  memories  of  the  spatial  order  of  things  suffer.  The  patient  can  not 
see  "in  his  mind's  eye,"  roads  that  were  formerly  familiar,  and  therefore 
easily  loses  his  way. 

Not  to  be  confused  with  disturbance  in  color  memories  (loss  of  color 
images)  or  with  disturbances  in  the  perception  of  colors  (color  blindnes — tested 
by  sorting  colors),  there  is  a  disturbance  in  naming  colors,  occurring  now 
with,  now  without  the  other  two  (inappositely  named  amnesic  colorblindness). 
In  this  case  the  patient  can  not  give  the  right  name  to  a  color,  just  as  he  can 
not  correctly  name  other  things.  (One  of  the  phenomena  in  amnesic  and  so- 
called  visual  aphasia,  that  shows  itself  especially  in  names  of  colors  and 
persons.) 

Amnesic  aphasia  is  usually  associated  with  mental  blindness  and  alexia, 
eventually  also,  with  agraphia. 

3.  Tactile  Agnosia  ("  Touch  Crippling"). — In  tactile  agnosia  the  patient 
does  not  recognize  the  object  touched  in  spite  of  the  presence  of  various  and 
sufficient  incoming  sensations  (perceptions  of  touch,  location,  movement, 
etc.).  For  example,  forms  are  not  recognized  (astereognosis).  Nor  do 
perceptions  of  moisture,  cold,  velvety  softness,  etc.,  lead  to  correct  conclusions. 

There  are  three  kinds  of  tactile  agnosia: 

1.  Either  separate  impressions  are  not  correctly  correlated,  though 
tactile  memories  are  retained,  that  is,  they  do  not  correctly  evoke  the  latter, 
or 

2.  Tactile  memories  are  lost,  or 

3.  The  association  between  them  and  the  visual,  auditory,  etc.,  memo- 
ries is  broken. 


544  DISEASES  OF  TEE  BRAIN 

Anatomically,  in  tactile  agnosia,  focal  injuries  exist  in  the  middle  third 
of  the  posterior  central  convolution,  or  behind  it,  in  the  parietal  lobe,  the  latter 
being  especially  concerned  in  the  third  associative  form. 

Not  to  be  confused  with  tactile  agnosia,  there  is  that  failure  to  recognize 
through  touch,  which  is  caused  by  severe  disturbances  in  sensation  and  by 
lesions  of  sensor}'  paths.  In  this  case  we  find  not  an  agnostic,  but  a  per- 
ceptive disturbance,  and  one  should  distinguish  also,  similarly  caused  dis- 
turbances in  form  recognitionas,  as  perceptive  astereognosis,  from  tactile 
agnosia  (see  page  506). 

4.  Agnosias  in  the  spheres  of  smell  and  taste  are  distinguished  with 
difficulty  from  the  corresponding  perceptive  disturbances  and  have  been 
little  studied. 

The  agnostic  (dissolutory)  disturbances  that  have  been  heretofore  dis- 
cussed, rest  on  the  fact,  that  the  members  of  the  series  of  ideas  essential  to 
cognition,  split  into  their  separate  sensory  components,  or  are  injured  by  the 
destruction  of  such  a  component;  for  instance,  the  optic.  Frequently,  how- 
ever, a  disturbance  in  recognition  is  caused  by  the  fact,  that  ideas,  intact  in 
their  sensory  elements,  are  combined  in  the  wrong  order,  that  perhaps  the 
connection  between  the  partial  conceptions  and  the  whole  concept,  or  the 
correct  association  of  cause,  purpose,  or  characteristics  with  an  object  is  lost, 
that  is,  those  associations,  necessary  to  recognition,  are  injured  that  do  not 
precisely  concern  the  connection  of  the  sensory  elements  {ideational  agnosia, 
counterpart,  and  frequently  companion  of  ideational  apraxia). 

Various  associative  and  attentional  disturbances  thus  affect  the  train  of 
ideas.  The  object  is,  for  example,  not  recognized,  because  a  " persevering" 
idea,  or  an  accidental  sensory  impression,  or  an  emerging  immaterial  side 
issue  interposes  and  turns  aside  the  train  of  ideas. 

These  ideational  agnosias  occur  especially  with  diffuse  mental  processes, 
furthermore  as  general  effects  of  focal  injuries,  hence  often  accompany  the 
focal  symptoms,  possibly  occur  even  as  direct  focal  symptoms.  They  present 
those  disturbances  in  cognition,  that  one  generally  characterizes  as  "psychic." 

If  optic,  tactile  and  acoustic  agnosia  are  associated,  we  have  total  agnosia, 
used  in  the  older  sense  of  total  asymbolia.  This  presupposes  such  extensive 
injuries,  large  lesions  in  both  temporal,  parietal,  and  occipital  lobes,  that 
memory  pictures  themselves  and  their  connection  with  understanding  are 
virtually  destroyed.     Then  there  is  entire  loss  of  comprehension. 

(c)  Apraxic  Disturbances 

1.  General  Remarks 

In  brain  diseases,  there  is  often  inability  to  move  the  limbs,  so  as  to  carry 
out  the  movements  intended  by  the  patient,  so  that  even  very  familiar  and 


APRAXIA  545 

thoroughly  learned  movements  can  not  be  made,  though  neither  palsy  nor 
ataxia  is  a  sufficient  cause. 

For  the  patient  can  occasionally  contract  all  muscles;  he  raises,  lowers, 
bends,  stretches  arm  and  hand  often  vigorously  and  once  in  a  while  executes 
complicated  movements,  and  is  thus  differentiated  from  the  paralytic;  but 
just  when  he  wishes  to  make  a  certain  movement  he  is  unable  to  do  it. 
Other  apraxics  can  carry  out  such  simple  purposive  movements,  but  the  still 
relatively  simple  movement  complexes,  such  as  greeting,  beckoning,  threat- 
ening, lighting  cigars,  sealing  letters,  pouring  out  water,  etc.,  they  can  not 
execute.  Objects  are  wrongly  used,  or  the  patient  stares  at  them,  being  at  a 
loss  as  to  what  to  do  with  them. 

The  limbs  then  can  not  be  made  to  serve  the  purposes  of  life.  As  in 
aphasia,  the  unparalyzed  muscles  of  tongue,  lips,  and  palate,  can  not  be  so 
directed  as  to  produce  the  word  in  mind,  the  apraxic  can  not  make  his  para- 
lyzed hand  execute  certain  movements.  The  expressive-aphasic  disturb- 
ances present  only  part  of  the  symptoms  of  apraxia,  just  as  the  receptive- 
aphasic  present  part  of  those  of  agnosia,  and  it  is  only  from  historical 
considerations  that  aphasic  disturbances  are  treated  separately. 

The  inability  to  perform  purposive  movements,  and  purposive  groups  of 
movements  in  the  every-day  affairs  of  life,  arises  from  very  different  causes. 

Partial  recollections  (i.  e.,  kinetic  or  optic)  for  combined  movements  may 
be  lost,  or  difficult  to  rouse,  or  the  connection  among  the  partial  components 
may  be  lost,  owing  to  an  interruption  in  the  fibres,  or  the  regular  outlet  of  the 
mental  process  that  prepares  complicated  movements,  step  by  step,  has  been 
affected  by  various  disturbances  (consisting  not  exactly  in  loss  or  splitting  of 
partial  memories)  of  mnemic,  associative,  attentional  kinds. 

The  apraxic  suffers  not  from  a  disturbance  of  those  lower  co-ordinations  of 
muscles,  known  as  ataxia,  which  is  caused  by  severe  sensory  disturbances. 

Taxia  so  regulates  the  working  together  of  muscles,  that  the  limbs, 
without  wavering,  on  the  shortest  path,  traverse  the  road  to  the  goal.  But 
which  road  is  to  be  traversed,  by  which  members  or  parts  of  members,  in  what 
co-ordination  or  sequence,  which  object  is  to  be  the  starting  point,  in  order  that 
the  desired  form  of  motions,  and  that  demanded  by  the  purposes  of  life, 
may  result,  and  how,  in  the  use  of  taxia,  all  necessary  simultaneous  and  suc- 
cessive innervations  shall  occur — in  these  questions,  taxia  says  nothing,  but 
praxia  rules. 

For  practical  differentiation  of  the  ataxic,  there  serves  among  other  tests, 
this — that  the  ataxic  performs  inexact  movements,  while  the  apraxic  (for 
exceptions  see  below)  performs  frequently  movements — quite  different  from 
those  desired,  that,  on  the  other  hand,  while  the  movements  of  the  apraxic 
do  not  correspond  to  the  intention,  they  appear  perfectly  co-ordinated,  viewed 
in  the  light  of  another  purpose.  He  may  write  the  wrong  letter,  that  is,  in 
itself  correct,  or  he  places  the  comb  behind  his  ear  with  the  muscular  move- 
35 


546  DISEASES  OF  THE  BRAIN 

ments  perfectly  suited  to  this  act.  Those  apraxics,  who  do  not  know  how 
to  handle  objects,  were  formerly  usually  classified  with  the  agnostics.  The 
word  apraxia  was  formerly  employed  when  some  one  used  an  object  incor- 
rectly. But  it  was  thought  that  he  did  so  from  mistaking  the  object  or  failing 
to  recognize  its  use.  As  one-sided  apraxics  prove,  a  man  can,  however, 
recognize  the  object,  know  its  use,  and  yet  be  unable  to  use  it. 

In  order  to  diagnose  apraxia,  it  must  be  proved  that  the  patient  recognizes 
the  object.  At  first  sight,  he  seems  to  be  mistaken,  as  when  he  makes  motions 
of  smoking  with  a  tooth-brush,  as  if  it  were  a  cigar. 

The  pre-essential  condition  of  purposive  movement,  as,  say,  the  lighting 
of  a  cigar,  is  that  the  details  of  this  movement  arise  internally,  in  correct 
sequence,  and  in  relation  to  the  right  object,  so  that  the  train  of  ideas  corre- 
sponds to  the  sequence  of  the  necessary  partial  movements,  and  their  relations 
to  objects  (in  very  frequently  performed  acts,  the  subconscious  cerebral 
equivalent  of  this  train  of  ideas).  This  is  the  ideational  "plan"  of  move- 
ment, which  determines  which  paths,  in  what  sequence,  are  to  be  run  over 
in  relation  to  what  object.  In  order  that  the  corresponding  movement  may 
be  actually  reached,  directions  corresponding  to  the  ideational  plan,  must  go 
to  the  motor  center  of  the  executing  limb,  i.  e.,  kinetic-innervational  memories 
must  be  awakened  which  release  the  real  innervation. 

If  the  ideational  "plan"  for  movement  is  wrong,  we  speak  of  ideational 
apraxia;  if  the  disturbance  is  only  in  the  transmission  of  ideational  plans  to 
the  special  kinematic  of  the  executing  limb,  we  speak  of  motor  apraxia  (in 
the  broader  sense). 

We  begin  with  the  latter. 

2.  Motor  Apraxia 

Two  subordinate  forms  are  to  be  distinguished: 

i.  Motor  apraxia  in  the  narrower  sense  or  ideo-kinetic  apraxia.  Here 
the  limb-center  with  its  kinetic-innervational  memories  is  itself  retained, 
but  through  breaks  of  countless  connections  with  the  other  brain  centers 
the  transmission  of  the  "plan"  of  the  movement  to  the  limb-center  is  hin- 
dered. The  disease  tears  asunder  ideation  (plan  of  movement)  and  limb 
kinematics.  It  must  be  assumed  that  in  the  sensory-motor  limb  center,  not 
only  the  seat  of  innervation,  and  the  substratum  of  synergies  is  laid,  but  also 
the  substratum  for  certain  much  practised  simple  movements,  the  building 
stones  of  complex  purposive  movements,  as  blowing,  whistling,  winking, 
writing  a  letter  of  the  alphabet,  etc. 

These  simplest  actions  do  not  need  directions  from  the  brain  as  to  every 
step  of  their  movement,  but  are  a  special  possession  of  the  limb  centers,  as 
are  the  synergies.  The  retention  of  this  special  possession  of  the  limb  center 
is  shown  clinically  by  the  fact,  that  occasionally  well-formed  movements  are 
executed,  i.  e.,  a  correct  letter  written,  hands  clasped,  etc.,  but  not  at  the 


MOTOR  APRAXIA  547 

proper  point,  not  when  the  patient  wishes  and  is  determined  to  do  it,  because  the 
necessary  co-operation  of  the  limb-center  with  the  brain  is  lacking. 

2.  If  on  the  other  hand,  from  a  lesion  in  the  limb-center  itself  that  does 
not  go  so  far  as  to  paralyze  it,  the  special  possession  of  the  limb-center  of  ki- 
netic memories,  be  injured,  the  seat  of  innervation  can  still  obey,  in  part,  the 
orders  of  the  brain;  but  the  loss  of  this  possession,  given  to  the  limb  by  long 
practice,  causes  all  movements  to  become  rough,  inexact,  uneconomic, 
like  those  of  a  man  who  is  attempting  them  for  the  first  time,  with  the  result 
that  such  movements  that  are  not  planned  in  the  rest  of  the  brain,  but  exist 
only  as  kinetic  memories  of  the  limb-center,  as  blowing  and  whistling,  can  not 
be  performed  at  all.  This  subordinate  form  of  motor  apraxia  may  be 
called  limb-kinetic  apraxia.1  In  so  far,  however,  as  co-operation  between 
limb-center  and  the  whole  brain  exists,  usually  through  the  mediation  of  this 
specialized  possession,  its  injury  will  cause  "derailments,"  which  resemble 
those  caused  by  breaks  in  the  paths  running  to  the  limb-center,  namely,  the 
ideo-kinetic. 
(a)  Clinical  Aspect  of  Ideo-kinetic  Apraxia  (Motor  Apraxia  Par  Excellence) 

It  is  confined  to  separate  limbs,  often  half  of  the  body.  (For  exceptions 
see  below.) 

A  train  of  simple  movements  is,  on  occasion,  quite  correctly  performed: 
the  patient  closes  his  hand  correctly  when  he  grasps  an  object,  but  is  unable 
to  do  the  same  thing  when  he  wishes  to  make  a  fist;  in  writing  he  writes  the 
wrong  letters,  though  each  is  correct  in  itself.  Beyond  this  occasional 
occurrence  of  correctly  executed  movements,  however,  even  the  very  simplest 
movements  fail,  when  the  stimulus  comes  from  this  part  of  the  brain  from 
which  the  limb-center  is  cut  off.  The  patient  can  not  copy  a  vertical  or 
horizontal  line  that  has  been  drawn  for  him,  nor  open  his  fist  when  he  is  told; 
more  complicated  movements,  pouring  out  water,  striking  matches,  etc., 
are  out  of  question.     The  following  kinds  of  false  reactions  occur: 

1.  Movements  that  resemble  no  purposive  movements,  such  as  beating 
the  hand,  spreading  the  fingers  (so-called  amorphous  movements). 

2.  Parapraxic  movements — winking  in  place  of  threatening,  touching  the 
ear  instead  of  the  nose,  etc. 

3.  The  movement  is  transferred  to  an  entirely  different  musculature — 
standing  straight  and  rigid,  in  place  of  extending  the  hand.  This  simulates 
real  loss  of  movement. 

4.  Often  motor  indecision  and  real  loss  of  movement  take  place.  In 
many  miss-reactions,  there  is  shown  a  strong  tendency  to  fixation  of  ideas 
(perseveration),  namely,  the  intended  movement  is  not  executed,  but  the 
preceding  one  repeated.  By  combination  of  such  "  persevering  "  movements 
with  components  of  the  new  purposive  movements,  very  curious  bastard 

1  This  conforms,  at  least  so  far  as  definition  is  concerned,  with  Meynert's  motor  asymboia. 


548  DISEASES  OF  THE  BRAIN 

formations  arise.  The  fixation  of  movements  is  probably  not  the  cause,  but 
the  result  of  the  right  movement  not  being  executed.  Ideo-kinetic  apraxia 
is  shown  even  in  imitative  movements. 

5.  Secondary  disturbances  of  the  nature  of  ideational  apraxia,  to  be 
described  below,  appear. 


(/?)  Clinical  Aspect  of  Limb-kinetic  Apraxia 

Since  very  extensive  focal  lesions  in  the  limb-center  lead  to  paralysis 
(which  conceals  the  loss  of  limb-kinetic  memories),  it  occurs  mainly  in 
discrete  processes  (arterio-sclerosis,  senile  atrophy,  progressive  paralysis). 
The  coarse  movements  are  awkwardly,  inexactly  performed  and  resemble 
those  in  cerebral  ataxia.  They  are  distinguished  from  them  in  this,  that 
for  many  more  delicate  actions,  sewing,  blowing,  whistling,  not  even  a 
tendency  towards  the  action  is  to  be  distinguished.  The  movement  shown 
in  these  actions,  is  not  only  an  ataxic  distortion  of  the  correct  one,  but  is 
wholly  lost,  or  bears  not  a  trace  of  resemblance  to  the  original  form.  Here, 
too,  secondary  ideational  disturbances  may  occur. 


3.  Ideational  Apraxia 

Here  manifold  disturbances  even  in  the  ideational  plan,  are  concerned — 
due  to  flaws  in  memory,  attention  and  association.  The  patient  omits 
parts  of  an  action,  performs  them  in  the  wrong  order,  or  makes  the  right 
movement  towards  the  wrong  object.  Such  a  strange  confusion  of  move- 
ments results,  that  the  patient,  e.  g.,  instead  of  striking  the  match,  places  it 
in  his  mouth,  next  to  a  cigar,  or  attempts  to  cut  off  the  end  of  the  cigar,  by 
inserting  it  between  the  two  parts  of  the  match  box.  In  sealing,  he  puts 
the  seal  in  the  fire,  and  then  impresses  it  on  the  wax.  The  errors  look  like 
results  of  absent-mindedness;  generally  the  association  bond  between 
correct  and  incorrect  movement  can  be  proved.  Single  movements  are 
quite  correct. 

Ideational  apraxia  appears  only  in  complicated  movements,  or,  at  least, 
grows  with  the  degree  of  complication.  Imitation  of  short  movements  is 
retained,  since  here  the  plan  of  movement  is  given  the  patient  from  without. 
Only  that  the  form  of  movement  does  not  spontaneously  occur  to  him. 
(If  the  latter  disturbance  predominates,  and  other  ideational  derangements 
appear  in  a  small  degree,  or  not  at  all,  as  is  often  the  case,  one  may  call  it 
amnesic  apraxia.)  It  is,  in  general,  not  confined  to  separate  limbs,  but 
affects  all  equally.  It  happens,  however,  that  in  a  slightly  motor-apraxic 
limb,  a  general  uncertainty  of  ideation  is  strongly  marked.  Ideational 
apraxia  is  very  often  associated  with  agnostic  disturbances. 


APRAXIA  549 

4.   The  Predominance  of  the  Left  Hemisphere  in  Controlling  Muscular  Actions 
and  the  Localization  of  Apractic  Disorders 

Heretofore,  it  has  been  assumed  that  the  plan  of  movement  rises  in  the 
entire  brain,  that  the  visual  images  in  both  hemispheres  and  the  kinetic 
centers  for  all  the  limbs  share  it  equally,  and  that  this  plan  is  transmitted 
to  the  center  of  the  acting  limb.  This,  to  a  certain  extent,  is  fiction.  It 
has  been  proved  that  the  special  kinetic  function  of  the  left  arm  center 
and  its  connection  with  the  rest  of  the  perception  centers  has  not  only  a 
peculiar  importance  for  the  movement  of  the  right  arm,  but  also  for  move- 
ment in  other  parts  of  the  body  and  most  surely  for  those  of  the  left  arm. 
Probably  the  arm  center  in  the  left  hemisphere  is  not  only  an  intermediate 
station  for  the  stimulation  of  the  arm  center  in  the  right  hemisphere  in 
purposive  movements,  but  the  kinetic  memories  of  the  arm  center  in  the 
left  hemisphere  are  an  almost  indispensable  prop  for  the  ideational  plan 
and  for  movements  of  the  left  hand. 

As  a  matter  of  fact,  we  find,  that  in  many  lesions  of  the  left  hemisphere, 
that  paralyze  the  right  hand  or  make  it  apraxic,  the  praxia  of  the  left  hand 
is  also  affected,  whence  it  is  clear,  that  the  direction  of  both  halves  of  the 
body  in  purposive  movements  is  assumed,  to  a  considerable  degree,  by  the 
left  hemisphere. 

The  left  hemisphere  predominates,  then,  as  in  speech,  so  also  in 
action  as  well,  even  if  not  in  similar  degree.     Focal  injuries  that  strike, 

1.  The  hand  center  in  the  left  hemisphere,  or 

2.  The  underlying  white  matter,  or 

3.  The  connection  of  the  hand  center  with  other  parts  of  the  brain, 
especially,  the  temporal,  parietal  and  occipital  lobes,  cause  in  the  majority 
of  people,  besides  the  effect  on  the  right  side  of  the  body  (in  1  and  2  mostly 
paralysis,  in  3  apraxia  of  the  right  arm)  the  following  dyspraxic  symptoms 
of  slighter  degrees  of  severity,  in  the  left,  unparalyzed,  upper  extremity: 

1.  Movements  from  memory  can  be  performed  only  in  a  curtailed 
fashion,  or  not  at  all :  the  patient  can  not  longer  differentiate  how  one  catches 
a  fly,  turns  a  hand-organ,  or  swings  a  cane,  etc.  Especially  he  can  not 
perform  correctly  expressive  movements,  as  threatening,  winking,  giving  a 
military  salute,  throwing  kisses,  making  a  long  face,  etc.  More  or  less 
distorted  movements  result,  and  many  perseveration  miss-reactions  occur. 
If  one  can  ascribe  all  this  to  mere  amnesia  (difiiculy  in  rousing  the  mem- 
ory of  the  meaning  and  the  word),  it  appears 

2.  That  the  patient  can  not  imitate  movements,  so  that  not  only  the 
spontaneous  evoking  of  movement  memories  is  assigned  to  the  left  hemi- 
sphere, but  a  direction  of  the  movements  of  the  left  hand  by  the  left  hemi- 
sphere must  be  maintained. 

3.  That  a  few  patients  manipulate  objects  wrongly,  even  with  the  left 


55° 


DISEASES  OF  THE  BRAIN 


hand.  The  majority,  on  the  other  hand,  can  when  they  see  and  feel  the 
objects,  by  the  help  of  optic-tactile-kinesthetic  signals  coming  from  the 
object,  perform  movements,  that  they  could  not  accomplish  from  memory 
alone. 


fitirnpol 


Fig.  156. — Horizontal  scheme  of  the  apraxic  disturbances.  L.H.,  left  brain  center  of  the  right 
hand.  R.H.,  right  brain  center  of  the  left  hand.  Co.,  C.p.,  C.t.,  cortical  origin  of  the  occipital, 
parietal  and  temporal  association  fibres  to  the  left  brain  hand  center.  The  corresponding  as- 
sociation paths  to  the  hand  center  in  the  right  brain  as  well  as  those  coursing  from  the  right 
himisphere  to  the  left  in  red  dashes,  to  point  out  their  subordinate  importance.  The  corpus 
callosum  connections  between  L.H.  and  R.H.  aremarked  by  two  full-drawn  red  lines.  The  blue 
line  which  at  the  end  of  the  arrow  deviates  from  the  plane  of  the  figure,  represents  the  projection 
fibres  of  L.H.  The  pathway  for  purposive  movements  of  the  right  hand  is  from  Co.,  C.p.,  C.t., 
over  L.H.  through  the  blue  line  into  the  anterior  horn  cells  of  the  cervical  cord.  For  purposive 
movements  of  the  left  hand  principally  from  Co.,  C.p.,  C.t.,  overL.  H.  through  the  corpus  callosum 
to  R.H.;  a  less  important  path  leads  through  the  red  dash  lines  to  R.H. 

1.  The  focus,  which  destroys  completely  L.H.:  paralysis  of  the  right  and  dyspraxia  of  the  left 
hand. 

1a.  Slighter  lesion  oiL.H.  which  does  not  lead  to  paralysis  but  destroys  the  mnemic  possession 
of  L.H.  only:  kinetic  apraxia  of  the  right  and  dyspraxia  of  the  left  hand.  2.  Paralysis  of  the 
right  and  dyspraxia  of  the  left  hand.  3.  (Focus  in  the  corpus  callosum)  dyspraxia  of  the  left  hand. 
4.  (Focus  behind  the  hand  center  in  the  parietal  lobe)  ideo-kinetic  apraxia  of  the  right  and  dys- 
praxia of  the  left  hand.  Foci  in  the  left  hemisphere  located  farther  backwards  and  diffuse  processes 
frequently  cause  ideatory  apraxia.  5.  Focus  in  the  capsula  causes  paralysis  of  the  right  hand  with- 
out causing  dyspraxia  of  the  left  hand.     Above  the  cut:  Stirnpol  =  frontal  pole. 

The  weight  of  evidence  and  anatomic  findings  indicate  that  the  right 
hemisphere  in  purposive  movements  of  the  left  arm  innervated  by  it,  receives 


APRAXIA  551 

through  the  cor  pis  callosum,  directions  from  the  left  hemisphere.  Extensive 
lesions  in  the  corpus  callosum  have  in  a  series  of  cases  made  the  left  hand 
dyspraxic. 

One  may  suppose,  then,  that  memories  of  well-learned  activities,  the  free 
plan  of  movements,  and,  finally  the  supervision  of  their  execution  are  pre- 
dominantly the  affairs  of  the  left  hemisphere  and  through  the  corpus  callosum 
are  transmitted  to  the  right. 

The  fact  that  apraxia  of  the  left  hand  in  focal  injuries  of  the  left  hemi- 
sphere is  usually  not  of  a  very  high  degree,  and  especially  often  spares  the 
manipulation  of  objects,  proves  that  the  right  hemisphere  is  not  wholly 
dependent  on  the  left  for  praxia,  but  has  a  certain  measure  of  kinetic  power 
peculiar  to  itself,  as  well  as  connections  with  the  entire  brain. 

Eupraxia,  then,  is  dependent  on  the  soundness  of  a  great  apparatus,  the 
main  part  of  which  is  the  arm  center  in  the  left  hemisphere.  If  this  is  wholly 
destroyed  (Fig.  156),  paralysis  of  the  right  arm  and  dyspraxia  of  the  left 
appear.  If  it  is  but  slightly  injured  (la)  limb-kinetic  apraxia  of  the  right 
arm  and  again  dyspraxia  of  the  left  appear.  If  the  white  matter  underneath 
the  hand  center  be  injured  (2),  the  right  arm  is  again  paralyzed,  and  the  left, 
because  the  corpus  callosum  fibres  are  broken  in  the  white  matter,  is  dys- 
praxic. If  in  the  parietal  lobes,  by  an  extensive  focal  injury,  the  connections 
of  the  arm  center  with  the  temporal  parietal,  and  occipital  lobes  and  the  right 
hemisphere  are  interrupted,  (4)  ideo-kinetic  apraxia  of  the  right  arm  appears, 
associated  with  slight  dyspraxia  of  the  left. 

Focal  injuries  still  farther  back,  in  the  hindmost  part  of  the  temporal, 
and  foremost  part  of  the  occipital  lobes,  as  well  as  diffuse  injuries  of  the  brain 
show  ideational  apraxia. 

If  only  the  corpus  callosum  is  interrupted  to  a  very  considerable  extent, 
(3)  the  left  hand  is  dyspraxic,  the  right,  is  neither  paralyzed  nor  dyspraxic. 
How  focal  injuries  in  the  right  hemisphere  by  robbing  the  arm  center  in  the 
right  hemisphere  of  the  nervous  impulses,  that  carry  the  directions  (and 
eventually  by  destroying  the  memory),  influence  praxia  of  the  left  hand,  has 
not  been  definitely  ascertained. 

Focal  injuries  that  destroy  the  projection  fibres  from  the  inner  capsule,  and 
farther  down  (5),  cause  no  apraxia  of  the  hand  on  that  side,  because  the 
corpus  callosal  fibres  are  not  affected.  So  that  only  supracapsular  focal  injuries 
lead  to  apraxia.  Hence  apraxia  is  a  characteristic  that  permits  us  to  dis- 
tinguish cortical  injuries  and  those  in  the  white  matter,  from  those  that  are 
more  deeply  laid,  in  the  capsules,  peduncles,  pons,  and  bulbar  region. 

A  phenomenon  has  been  described  under  the  name  mind  palsy  which 
stands  between  actual  paralysis  and  apraxia :  a  limb  can  not  be  moved  at  will, 
or  only  with  great  difficulty,  but  shows,  through  movements,  that  are  per- 
formed under  peculiar  circumstances  that  it  is  not  really  paralyzed,  and  by 
their  accuracy,  that  it  is  not  apraxic.     It  is  only  a  matter  of  great  difficulty  in 


552  DISEASES  OF  THE  BRAIN 

innervation.  Since  the  words  "mind  palsy"  (Seelenlaehmung)  are  used  in 
very  different  senses,  one  should  call  this  phenomenon  "volition  palsy." 

Whether,  and  to  what  extent,  the  left  frontal  brain  lobe,  participates  in 
praxia,  is  as  yet  uncertain. 

The  left  parietal  lobe  is  of  the  greatest  importance  for  apraxia.  Exten- 
sive focal  injuries  in  the  left  hand  center,  itself  (central  convolutions),  and 
in  its  white  matter,  paralyze  mostly  the  right  hand  (la  is  a  rare  case) ;  apraxia 
is  concealed  in  the  right,  and  shows  itself  only  in  quantitatively  slighter 
disturbances  of  the  left  hand.  The  lesion  of  the  parietal  lobe,  on  the  con- 
trary, causes  apraxia  of  the  right  hand  to  appear  clearly.  Lesions  of  the 
corpus  callosum  have  considerable  influence  only  as  regards  the  praxia  of 
the  left  hand.  So  the  left  parietal  lobe  is  not  at  all  the  center  for  praxia  in 
the  sense  that  there  alone  purposive  movements  are  "made,"  but  in  the 
sense  that  it  is  that  place  in  the  brain,  in  which  lesions  can  interrupt  in  great 
number,  and  with  comparative  isolation,  connective  paths  most  necessary 
for  praxia. 


COLUMBIA  UNIVERSITY  LIBRARIES 

This  book  is  due  on  the  date  indicated  below,  or  at  the 
expiration  of  a  definite  period  after  the  date  of  borrowing, 
as  provided  by  the  rules  of  the  Library  or  by  special  ar- 
rangement with  the  Librarian  in  charge. 

DATE  BORROWED 

DATE  DUE 

DATE  BORROWED 

DATE  DUE 

/f       ft,. 

' 

.     -.   ■. 

C2SU  I401HIOO 

Base  H^l^o.  '20. 


SC341 
Curs  cfoiarm 


0932 


Base  Hospital  No.  20. 


'.■"''■  ' ' '     ■  - '. ','. . : '. ' . 


